TAPVC A CASE STUDY
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Transcript of TAPVC A CASE STUDY
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CASE STUDY TAPVC REPAIRJAYA BABU SSTAFF NURSE CHICU
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• 50 DAY OLD BABY
• FIRST CHILD ,FULL TERM NORMAL DELIVERY
• BIRTH WEIGHT -2.7 Kg
• H/o bronchopneumonia at the age of 1 month
• Based on investigations and clinical symptoms detected to have SUPRACARDIAC TAPVC
• Underwent TAPVC rerouting
PATIENT PROFILE
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TAPVC TOTAL ANOMALOUS PULMORAY
VENOUS CONNECTION
NO DIRECT CONNECTION BETWEEN ANY PULMOARY VEIN AND LEFT ATRIUM
ALL THE PULMONARY VEINS CONNECT TO RIGHT ATRIUM OR ONE OF ITS TRIBUTARIES
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TYPES
SUPRACARDIAC
45%
CARDIAC
25%
INFRACARDIAC
25%
MIXED
5%
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SUPRACARDIAC TAPVC
PULMONARY VEINS CONVERGE
BEHIND THE LEFT ATRIUM
COMMON ANOMALOUS
VERTICAL VEIN
LEFT INNOMINATE VEIN
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CARDIAC TAPVC
The pulmonary venous confluence drains into the coronary sinus
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INFRACARDIAC TAPVC
The pulmonary venous confluence
drains into a descending vertical
vein through the diaphragm into the
portal vein or ductus venosus.
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MIXED TAPVC
It can involve any or all components
of the previous three types.
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PATHOPHYSIOLOGY
• COMPLETE LA PV DISCONNECTION
• PV BLOOD GOING INTO RA
• AN INTRAATRIAL COMMUNICATION USUALLY ASD OR PFO
• DEGREE OF CYANOSIS DEPEND ON AMOUNT OF PULMONARY BLOOD FLOW
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PATHOPHYSIOLOGY…..
INCREASED PULMONARY BLOOD FLOW
PULMONARY HYPERTENSION
MUSCULARITY OF THE PULMONARY
ARTERIOLES
LABILE PULMONARY
VASCULAR RESISTANCE
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OBSTRUCTION TO PULMONARY VENOUS DRAINAGE
SUPRACARDIAC 65%
CARDIAC 17-20%
INFRACARDAIC 100%
SUPRACARDIAC
STENOSIS OF THE LEFT VERTICAL VEIN
COMPRESSION OF VERTICAL VEIN BETWEEN PULMONARY ARTERY AND
LEFT MAIN BRONCHUS
ANATOMIC VISE
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OBSTRUCTED TAPVC …
AT THE JUNCTION WHERE THE
COMMON VEIN JOINS THE
CORONARY SINUS
AT THE ORIFICE OF THE
CORONARY SINUS
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OBSTRUCTED TAPVC …STENOSIS WHERE IT ENTERS PORTAL OR HEPATIC VEIN
OR DUCTUS VENOSUS
COMPRESSION PASSING
THROUGH THE DIAPHRAGM
HIGH RESISTANCE PATHWAYS
IMPOSED BY FLOW THROUGH HEPATIC
MICROVESSELS
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CLINICAL MANIFESTATIONS
UNOBSTRUCTED TYPE
ASYMPTAMATIC
ONLY MILD CYANOSIS
FAILURE TO THRIVE
DYSPHONIA
SLIGHT HEPATOMEGALY
CARDIOMEGALY
SYSTOLIC EJECTION MURMUR
SNOWMAN SIGN IN C-XRAY
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OBSTRUCTED TYPESYMPTAMATIC WITHIN FEW
HOURS AFTER BIRTH
• MARKED RESPIRATORY DISTRESS WITH CYANOSIS
• FEATURES OF PULMONARY OEDEMA
• PROGRESS TO CARDIOGENIC SHOCK
• XRAY SHOWS MARKED VENOUS CONGESTION WITH A GROUND GLASS APPEARANCE AND NO CARDIOMEGALY
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Medical management
UNOBSTRUCTED TYPE
• COMPENSATING RIGHT HEART FAILURE
– INOTROPIC SUPPORT
– DIURESIS
• OBSTRUCTED TYPE
LIMITED ROLE
INTUBATION AND HYPERVENTILATION
CORRECTION OF ACIDOSIS
OBSTRUCTED TAPVC IS A TRUE SURGICAL EMERGENCY!!!!!!!!!
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Interrupt the connections
with the systemic venous
circulation
An unobstructed communication
between the pulmonary venous confluence and the
left atrium
close the atrial septaldefect
SURGERY
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SC TAPVC SURGICAL TECHNIQUE
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INFRACARDIAC TAPVC REROUTING
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POSTOPERATIVE COMPLICATIONS
• PULMONARY OEDEMA
• PULMONARY HYPERTENSIVE CRISES
• PHRENIC NERVE DAMAGE
EARLY COMPLICATIONS
• PULMONARY VENOUS OBSTRUCTION
• ANASTOMOTIC STRICTURE
• PULMONARY VEIN STENOSIS
LATE COMPLICATIONS
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KEY POINTS IN POSTOPERATIVE CARE
Maintain adequate cardiac output
Keep the left atrial pressure as low as
possible
Prevention and management of PAH
crises
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PA PRESSURE MONITORING
• PAP should be than less than 2/3 rd of the systemic pressure
• In PAH CRISIS, PAP becomes suprasystemic.
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Rapid increase in PVR
PAP exceeds systemic blood pressure (BP).
Decreased cardiac output, hypoxia,
Decrease in pulmonary blood
flow
PAH CRISIS
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RECOGNITION OF PAH CRISIS
TACHYCARDIA
HYPOTENSION
High PAP
ABRUPT DESATURATION
BRADYCARDIA
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PREVENTION
Correct metabolic acidosis.
Hyperventilate
sedation
Attenuate noxious stimuli
Support cardiac output.
pulmonary vasodilators.
100% oxygen.
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POSTOPERATIVE COURSE
• SURGERY: Primary sutureless repair by right lateral approach.
• Received in ICU with stented sternum and PA line in situ.
• On ventilator with FiO2 80%– SIMV 32/TV-30/PEEP-4
– Ph-7.45 / Pao2-99.4 / Paco2-34.4 Lactate-0.9
• Stable hemodynamic– ABP:109/71 PAP:35/23(29) CVP:7
• Sedated with Morphine
• Milrinone 0.5mcg/kg/mt
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POSTOPERATIVE COURSE Contd…• Lasix infusion 2mg/kg/day• Sternum closed after 24 hours• Extubated next day and put on NIV• Post extubation maintained stable hemodynamics and PAP within
normal range• Milrinone tapered off
• On th 4th POD ,PAP :69/38(50) ABP:54/32(41)– Spo2-93% ABG:7.35/ 68/44 – Lactate: 2.1– PERIPHERAL TEMP: 28.6 ,Urine Output –Nil for 3 hours
• Management:– Reintubated– Noradrenaline and Dopamine infusion started– Sildenafil infusion started in PA Line– PD started
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• Improved clinically– ABP Improved
– PAP:24/20(22)
– Spo2 :100%
– Urine output 10ml/hr
• PD discontinued after 48 hours
• Supports were tapered off.
• Extubated again 48 hours of ventilation
• Put on NIV and O2 mask alternatively..
• Improving clinically
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• THE POSTOPERATIVE PERIOD IS CHALLENGING….
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