Insert subject into PH Professional Network: 2015 title...
Transcript of Insert subject into PH Professional Network: 2015 title...
![Page 1: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/1.jpg)
PH Professional Network: 2015
Nightmares in Pediatric PH
Russel Hirsch, M.D.
Director , Pulmonary Hypertension Service
The Heart Institute
Cincinnati Children’s Hospital Medical
Center
Cincinnati, Ohio
Insert subject intotitle master
Patricia Lawrence, PNP
Pediatric Nurse Practitioner
Children’s Healthcare of Atlanta
Atlanta. Georgia
![Page 2: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/2.jpg)
Disclosures
Dr. Russel Hirsch:
• Proctor, St. Jude Medical
• Off-label use of approved drugs
Patricia Lawrence:
• Research support from Eli Lilly
• Off-label use of approved drugs
This continuing education activity is managed and accredited by
Professional Education Services Group in cooperation with the
Pulmonary Hypertension Association. Neither PESG, nor PHA, nor
any accrediting organization support or endorse any product or
service mentioned in the is activity.
PESG and PHA staff has no financial interest to disclose.
Commercial Support was not received for this activity.
![Page 3: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/3.jpg)
Learning Objectives
At the conclusion of this activity, the participant will be able to:
1. Recognize risks associated with PH therapy and how PH therapy can
be harmful in certain patient populations
2. Describe solutions for preventing medication errors in the outpatient
setting for patients on parenteral PH treatments
3. Discuss potential side effects that impact a patient’s quality of life
![Page 4: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/4.jpg)
Overview
• Every diagnosis of Idiopathic PH in a child is a
nightmare
• Cases we will discuss will highlight:– Difficulties in diagnosis
– Complexity of management
– Adverse effects of medications
– Impact of sepsis
– Autonomy and ethical aspects in decision making
![Page 5: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/5.jpg)
KH, 12 year old female
• Asymptomatic, normal activities– Competitive basketball player
• Cleaning horse stall and kicked in the belly
• ER evaluation with severe abdominal pain /
hematuria
• MRI– Renal contusion
– Multiple hepatic lesions (AVM vs. hepatic nodules)
– Dilated IVC
![Page 6: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/6.jpg)
KH: Initial Evaluation
• No cardiac symptoms
• BP 132/60
• Sternal heave / Palpable 2nd heart sound
• Loud, wide split S2
• 3cm hepatomegaly, mild tenderness
![Page 7: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/7.jpg)
KH: Baseline Studies
![Page 8: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/8.jpg)
KH: Baseline Echo Images
![Page 9: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/9.jpg)
KH: Baseline MRI Images
![Page 10: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/10.jpg)
KH: Baseline CC
BL
30%
FiO2
iNO 80ppm /
FiO2 80%
Rp
(WU x m2)
9.1 9.8
Rs
(WU x m2)
13.5
CI
(l/min/m2)
5.4 3.68
Mean PA
Pressure
(mmHg)
52 50
Mean RA
Pressure
(mmHg)
7
![Page 11: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/11.jpg)
KH: Course
• Open liver biopsy during cardiac catheterization,
and hepatic vein injections– Focal nodular hyperplasia with malignant potential
– Portal venous malformation with Abernathy-type physiology
![Page 12: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/12.jpg)
KH: Course
• Medications commenced as out-patient– Oral Sildenafil
– Inhaled treprostinil• Dose increased gradually to maximum of 9 breaths q4h
• Initial occasional dizziness, but then well tolerated
– Bosentan contra-indicated
• At 3 months– Asymptomatic
– Compliant
– Liver transplant evaluation in progress• Parents somewhat reticent at this stage
![Page 13: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/13.jpg)
KH: Follow-Up Cath: 5 Wks
BL
30%
FiO2
Follow-Up
(5 weeks)
Rp
(WU x m2)
9.1 4.95
Rs
(WU x m2)
13.5 11.73
CI
(l/min/m2)
5.4 5.45
Mean PA
Pressure
(mmHg)
52 42
Mean RA
Pressure
(mmHg)
7 7
![Page 14: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/14.jpg)
KH: Follow-Up: 9mo
• No change in symptoms
• Echo Deterioration– RV dilated / function worse
– Increased TR Doppler jet velocity
– Increased septal flattening
• Deterioration in hepatic parameters– Albumin decreased
– GGT increased
– Ammonia increased
![Page 15: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/15.jpg)
KH: Follow-Up Cath: 10mo
BL
30%
FiO2
Follow-Up
(5 weeks)
Follow-Up
(10 mo)
Rp
(WU x m2)
9.1 4.95 7.8
Rs
(WU x m2)
13.5 11.73 8.0
CI
(l/min/m2)
5.4 5.45 4.6
Mean PA
Pressure
(mmHg)
52 42 52
Mean RA
Pressure
(mmHg)
7 7 9
![Page 16: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/16.jpg)
KH: Clinical Adjustments
• Broviac central line placed– After much family discussion
• Converted from inhaled to IV treprostinil– Titrated up to a dose of 23ng/kg/min at time of DC
• Considerable more discussion in regard to liver
transplantation
![Page 17: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/17.jpg)
Survival: Portopulmonary Hypertension
• REVEAL Registry data*• 5 yr survival 40% in subjects with PoPH versus 64% IPAH/FPAH
• Review of Mayo Data (1994-2007)**• 74 PoPH patients
• No treatment (19 pts): 5 yr survival 14%
• Pulm Vaso-dilator (43 pts): 5 yr survival 45%
• Liver Transplantation***• mPAP > 50mmHg - 100% liver transplant mortality
• mPAP < 35mmHg – 100% liver transplant survival
* Krowka et.al. Chest 2012;141:906-915
**Swanson et.al. AmJTransplant 2008;8:2445-2453
***Krowka et.al.Liver Transpl 2000;6:443-450
![Page 18: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/18.jpg)
On-Going ManagementBL
30%
FiO2
Follow-
Up
(5
weeks)
Follow-
Up
(10 mo)
Follow-
Up
14 mo)
Rp
(WU x
m2)
9.1 4.95 7.8 6.59
Rs
(WU x
m2)
13.5 11.73 8.0 12.44
CI
(l/min/m2)
5.4 5.45 4.6 5.3
Mean PA
Pressure
(mmHg)
52 42 52 49
Mean RA
Pressure
(mmHg)
7 7 9 9
![Page 19: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/19.jpg)
On-Going Management
• Regular clinic visits
• Further up-titration to 54ng/kg/min
• Generally feeling well, no complaints
• Full-time at school
• Intense conversation re liver transplantation
![Page 20: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/20.jpg)
On-Going Management
BL
30%
FiO2
Follow-
Up
(5
weeks)
Follow-
Up
(10 mo)
Follow-
Up
14 mo)
Follow-
Up
(18 mo)
Rp
(WU x m2)
9.1 4.95 7.8 6.59 6.1
Rs
(WU x m2)
13.5 11.73 8.0 12.44
CI
(l/min/m2)
5.4 5.45 4.6 5.3 6.1
Mean PA
Pressure
(mmHg)
52 42 52 49 51
Mean RA
Pressure
(mmHg)
7 7 9 9 9
![Page 21: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/21.jpg)
Clinical Course
• Presented to local ER 4 weeks post cath– Low grade fever x 2 days
– Subtle loss of appetite
– Diagnosed with UTI, treated nitrofurantoin
– Fever resolved
• Called PH office 3 weeks later– 7.5Kg weight loss
– Intermittent diarrhea
– Malaise
– Febrile
![Page 22: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/22.jpg)
Acute Presentation
• Unwell appearing
• Bloated
• Pale
• Febrile to 38.7°C
• Pulse 128/min
• BP 135/80
• RR 20/min
![Page 23: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/23.jpg)
Acute Presentation
• Mild peripheral edema
• Hyperdynamic precordium
• Prominent sternal heave / palpable P2
• Loud S2 with, with wide split P2
• 3/6 holosystolic murmur at tricuspid area
• Liver unchanged / no ascites
• Clear lung fields
![Page 24: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/24.jpg)
CXR
First Presentation Current Presentation
![Page 25: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/25.jpg)
New Studies
![Page 26: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/26.jpg)
New Study
First Presentation Current Presentation
![Page 27: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/27.jpg)
New Studies
![Page 28: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/28.jpg)
Presumed Diagnosis: Endocarditis
• Blood Cultures– Positive for Streptococcus Mitis (CVC as well as peripheral cultures)
• CRP: 10.9 (< 0.3)/ ESR: > 40
• CBC / chemistries / liver enzymes stable
• Mild increase in INR
![Page 29: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/29.jpg)
Subsequent Course
• Commenced on Ceftriaxone
• Over course of 6 day stay– Initial mild clinical improvement
– Broviac removed / PICC line inserted once cultures negative
– Fever resolved
– Appetite initially improved, then deteriorated
• Just prior to discharge– Sleeping more
– Mild peripheral edema
– Further loss of appetite
– Echo deterioration
![Page 30: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/30.jpg)
Echo Prior to Discharge
![Page 31: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/31.jpg)
Options……
• Repair / replace the tricuspid valve– Almost certain RV failure with need for mechanical support
? Endpoint
• Repair / replace the tricuspid valve with
transition to Novalung? Endpoint
• Repair / replace the tricuspid valve with
transition to Novalung, and list for lung and liver
transplant
![Page 32: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/32.jpg)
The Family’s Decision
• To go home…..– Neurologically and behaviorally intact teenager
– Previously perfectly well
– Curable PH with liver transplant with excellent prognosis
– No part in decision making
– Patient was not consulted
– Parents did not wish to have her informed of the issues or the prognosis
– Parents did not wish to have palliative care involved
![Page 33: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/33.jpg)
Course
• Discharged on stable medications– IV Treprostinil 54ng/kg/min via PICC
– Sildenafil
– Lasix
– Antibiotics (to complete 6 week course)
– Analgesia
– Nasal cannula oxygen
• Over first 2-3 weeks– Shortness of breath
– Peripheral edema
– Bloated belly
– Intermittent chest pain
– Increased sleepiness and lethargy
![Page 34: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/34.jpg)
Course
• Activities– Family road trip to Montreal
– Make-a-Wish to Hawaii to swim with dolphins• (needed to be supported in the water)
• Completed antibiotics 5 weeks post discharge
• Family decided to forgo follow-up appointment at
that time
………..then……
• 8 weeks post discharge, mother called to report
that she seemed to be doing better
![Page 35: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/35.jpg)
Follow-Up Visit at 10 Weeks
• Felt well, no complaints
• Normal appetite
• No shortness of breath
• No further swelling of her legs or belly bloating
• No longer using supplemental O2
• No analgesia
• Wanted to go back to school
• Resumed riding her horse
![Page 36: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/36.jpg)
Follow-Up Visit at 10 Weeks
• Exam:– P 90/min; BP 120/70; O2 Sat 100%
– Peripheries warm / normal pulses
– Quite precordium
– Mild prominence of the S2, with variable split
– Quiet 1/6 holosystolic murmur at the left upper sternal border
– Clear lung fields
– Soft belly; no masses
![Page 37: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/37.jpg)
Follow-Up Echo
![Page 38: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/38.jpg)
Follow Up Echo
![Page 39: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/39.jpg)
Wow!!!!!!!
![Page 40: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/40.jpg)
Where to from here….?
• Scheduled for cath…….rescheduled
• Maintained on same medication
• Credibility of care givers a major family issue
• Pathophysiology of her course is remarkable
• Teenager patient autonomy
![Page 41: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/41.jpg)
Children’s Healthcare of Atlanta
Case #2
![Page 42: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/42.jpg)
Children’s Healthcare of Atlanta
CS, a 10 year old male with
an unusual history
• Presented to our PH team in December 2012 after admission to PICU for hypoxia and shortness of breath
• Cardiology fellow saw patient night of admission
• “He’s had a normal echo in the past, but let’s just do another one”
• Echo findings led to PH inpatient consult
![Page 43: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/43.jpg)
Children’s Healthcare of Atlanta
Chart review and detailed history
![Page 44: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/44.jpg)
2011
Syncope
2/2011
Nl head CT
ref to
Cardiology
Normal
EKG & Echo
March
2011
Normal
exercise test
March 2011
![Page 45: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/45.jpg)
2011
Syncope
2/2011
Nl head CT
ref to
Cardiology
Normal
EKG & Echo
March
2011
Syncope
4/25/2011
Borderline
QTc
-Cardiology
Consult-
Event
Syncope
4/30/2011
with event
monitor
Syncope
5/7/2011Tilt
table
Admission
![Page 46: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/46.jpg)
Children’s Healthcare of Atlanta
Chart review and detailed history
• Referral and visits to Neurology on 5/2011 and
8/2011
• Exam and EEGs all suggestive of neurocardiogenic
syncope
• Felt to have ‘reactive autonomic nervous system’
![Page 47: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/47.jpg)
2011
1
2
3
4
Syncope # 5
11/2011
![Page 48: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/48.jpg)
Children’s Healthcare of Atlanta
Chart review and detailed history
• 2/2012 - Referral to endocrinology
– No endocrinologic explanations for syncopal episodes
![Page 49: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/49.jpg)
2011 2012
5/2012 11/2012 Admission
5 Syncopal
Episodes
![Page 50: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/50.jpg)
2011 2012
5/2012 11/2012
5 Syncopal
Episodes
12/7/12 Admission
![Page 51: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/51.jpg)
Children’s Healthcare of Atlanta
CS meets the PH team after 12/7/12 admission
for hypoxia
![Page 52: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/52.jpg)
Children’s Healthcare of Atlanta
CS meets the PH team after 12/7/12 admission
for hypoxia
Tests included:
• Walk test distance 378, desaturation to 85%
• PSG without OSA, + nocturnal hypoxemia
• Pulmonary Function Tests
![Page 53: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/53.jpg)
Children’s Healthcare of Atlanta
12/2012 Admission
CXR
• CXR - interstitial lung
disease vs pulmonary
hemorrhage
![Page 54: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/54.jpg)
Children’s Healthcare of Atlanta
12/2012 Admission
Chest CT
![Page 55: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/55.jpg)
Children’s Healthcare of Atlanta
Abnormal vs Normal Chest CT
![Page 56: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/56.jpg)
Children’s Healthcare of Atlanta
12/2012 Admission
Echocardiogram
![Page 57: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/57.jpg)
Children’s Healthcare of Atlanta
PH “Crisis”
• After getting emotional & angry with parents after
learning he may not go home for a few days
![Page 58: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/58.jpg)
Children’s Healthcare of Atlanta
12/2012 Admission
cardiac catheterization
Rest iNO + O2
CVP 11
mPAp 52
PVR 15.9 9.1
Wedge 10
CI
• Attempted bronch in cath lab, discontinued
• Extensive pruning of distal small branches
• Started on tadalafil with no evidence
of pulmonary edema
Rest iNO + O2
CVP 11 11
mPAp 52 43
PVR 15.9 9.1
Wedge 10 11
CI 2.6 3.5
![Page 59: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/59.jpg)
Children’s Healthcare of Atlanta
Several PH “crises” at home
• 12/2012 and 1/2013 requiring short admissions
• Usually occurred after emotional outburst or activity
• Saturations would often dip to mid 70s
• lasix started, tadalafil stopped and restarted at smaller dose
![Page 60: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/60.jpg)
Children’s Healthcare of Atlanta
Lung biopsy
• 1/29/2013- Confirmed
PVOD
– Arrest after
procedure requiring
intubation, pressors
– Pulse steroids started
![Page 61: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/61.jpg)
Children’s Healthcare of Atlanta
Photos of lung surface
at time of biopsy
![Page 62: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/62.jpg)
Children’s Healthcare of Atlanta
Photos of lung surface
at time of biopsy
![Page 63: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/63.jpg)
Children’s Healthcare of Atlanta
Normal lung surface
![Page 64: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/64.jpg)
Children’s Healthcare of Atlanta
Pulmonary Veno-occlusive Disease
Normal Abnormal
![Page 65: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/65.jpg)
Children’s Healthcare of Atlanta
PVOD
• Overlap of PVOD and PCH
• Causes of PVOD unknown
• 5-10% of cases diagnosed with idiopathic PAH
• Wide age range but primarily in children and adults
• Median survival ~ 2 years from diagnosis
• Symptoms include cough, hypoxia and progressive
dyspnea
![Page 66: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/66.jpg)
Children’s Healthcare of Atlanta
Pediatric PVOD – a retrospective analysis
![Page 67: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/67.jpg)
Children’s Healthcare of Atlanta
PH clinic 4/2013
• Echo
• Walk test
• PH staff witnessed crisis immediately after walk
test
![Page 68: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/68.jpg)
Children’s Healthcare of Atlanta
PH crisis in clinic after walk
• Sats to 70s
• HR to 140
• Flash pulmonary
edema with crackles
which resolved within
1 hour
![Page 69: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/69.jpg)
Children’s Healthcare of Atlanta
Admission after PH crisis 4/2013
• Admission – started Imatinib
– PACT team consult
– Family not interested in lung transplant
– Hospice
– Patient not consulted about wishes (11 years old at time)
![Page 70: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/70.jpg)
Children’s Healthcare of Atlanta
Imatinib for PVOD
![Page 71: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/71.jpg)
Children’s Healthcare of Atlanta
Clinical improvement within weeks
CT one year later
![Page 72: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/72.jpg)
Children’s Healthcare of Atlanta
Chest CT comparison
![Page 73: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/73.jpg)
Children’s Healthcare of Atlanta
Echocardiogram at time of repeat Chest CT
![Page 74: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/74.jpg)
Children’s Healthcare of Atlanta
Subsequent visits
• Stable echos
• Complaint of ‘breathing bothers me’
• Steroid weans
• Main complaint of abdominal pain
• Constipation
![Page 75: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/75.jpg)
Children’s Healthcare of Atlanta
Interim Data
Red – TR jet
Gold –walk
Blue - BNP
![Page 76: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/76.jpg)
Children’s Healthcare of Atlanta
Follow up cardiac catheterization
12/2012 1/2015
CVP 11 9
mPAp 52 48
PVR 15.9 11.7
Wedge 10 11
CI 2.6 3.14
-Increased tadalafil
-Later started calcium channel blocker
![Page 77: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/77.jpg)
Children’s Healthcare of Atlanta
Subsequent visits
• Occasional complaints of chest pain and difficulty with
breathing but no change in vital signs, echo
• No syncopal episodes since 12/2012 since starting on
tadalafil
• No PH crises from 4/2013 when Imatinib started
• Patient not informed of diagnosis or prognosis
![Page 78: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/78.jpg)
Children’s Healthcare of Atlanta
And then...a PH crisis
April 2015
• Increased CCB
• Another PACT team visit
• Steroid burst
![Page 79: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/79.jpg)
Children’s Healthcare of Atlanta
PH clinic 7/22/15
• Seen for complaints of chest tightness, increased
oxygen requirement
• Echo stable
• BNP less than 10
• Normal physical exam with splitting of S2
• Long discussion about family’s wishes given ‘return’ of
PH crises
![Page 80: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/80.jpg)
Children’s Healthcare of Atlanta
And then…..a syncopal episode
Admitted 8/13/15
![Page 81: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/81.jpg)
Children’s Healthcare of Atlanta
Clinical Data
• BNP normal
• LFTs normal
• CXR improved 12 hours later
• O2 requirement returned to normal
![Page 82: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/82.jpg)
Children’s Healthcare of Atlanta
Most recent clinical data
Red – BNP
Gold –TR jet
Blue - PVR
![Page 83: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/83.jpg)
Children’s Healthcare of Atlanta
8/13/15 Admission for syncopal episode
• Family devastated by ‘return’ of syncope
• Imatinib increased
• Discontinued amlodipine
• Palliative care team involvement
• Discussions around lung transplant resumed
• Family shared diagnosis and prognosis with patient
![Page 84: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/84.jpg)
Children’s Healthcare of Atlanta
Hot off the presses
• Clinic visit 9/16/15
• TR 120
• 6 minute walk distance 308
• BNP 70
• Admitted to restart amlodipine, monitor
• ? Intercurrent illness?
• Family has appointment with lung transplant center for
evaluation
![Page 85: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/85.jpg)
Children’s Healthcare of Atlanta
What next?
• Additional PAH therapy?
• Atrial Septostomy?
• Lung transplant?
![Page 86: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/86.jpg)
2011 2012 2013 2014 2015
5 Syncopal
Episodes
3 Syncopal
Episodes
1 Admit 4 Admits 5 Admits
1 Syncopal
Episode
1 Admit 3 Admit
![Page 87: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/87.jpg)
Children’s Healthcare of Atlanta
Pulmonary Hypertension Team
at CHOA
• Amanda Brown, PNP
• Anna Burnett Gay, PNP
• Jeryl Huckaby, RRT
• Usama Kanaan, MD
• Nikhil Chanani, MD
• Dawn Simon, MD
![Page 88: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/88.jpg)
Pulmonary Hypertension Team
at
CCHCM
• Michelle Cash
• Alison Cress
• Lisa Burns
• Jenna Faircloth
• Kathy Gosney
• Bill Nichols
• Katie Lutz
• Mike Pauciulo
• The nurses on A6C and
in the CICU and NICU
• Fellows who assist with
consultations
![Page 89: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/89.jpg)
Children’s Healthcare of Atlanta
What questions do you have?
![Page 90: Insert subject into PH Professional Network: 2015 title ...pha.files.cms-plus.com/Symp2015_B_NightmaresPediatric.pdfPulmonary Hypertension Association. Neither PESG, nor PHA, nor any](https://reader033.fdocuments.in/reader033/viewer/2022041420/5e1e414662e9cf6a9e7ba9d7/html5/thumbnails/90.jpg)
Children’s Healthcare of Atlanta
Obtaining CME/CE Credit
If you would like to receive continuing education
credit for this activity, please visit:
http://pha.cds.pesgce.com