Dr. Lt. Col. Md Abdul Hannan MBBS, FCPS (Surgery),

FCPS (Cardiovascular Surgery) Fellowship in CTVS (NUH, Singapore) Classified Cardiovascular Surgeon

CMH Dhaka, BANGLADESH

Follow up of a case : Elective replacement of the aortic root

in Marfan Syndrome by Composite valve graft with Mechanical valve (Modified Bentall Procedure) Lt Col MA Hannan1, Brig Gen Musa Khan2, Maj Gen Munshi MM Rahman3, Michael G. Caleb4 , C N LEE5

Marfan syndrome – An autosomal dominant inherited disorder of connective tissue, Mutation of FBN1 gene on chromosome 15 abnormal fibrillin protein, characterised by loss of elastic tissue, affects numerous body systems, including

the musculoskeletal, cardiovascular, neurological, and respiratory systems, the skin and eyes.

INTRODUCTION

Antoine Bernard-Jean Marfan (June 23, 1858 – February 11, 1942), a French pediatrician.

In 1896, Marfan described a

hereditary disorder of connective tissue in a 5 yr old girl with disproportionately long limbs that later became to be known as Marfan syndrome

HISTORY

Clinical Manifestations Diagnostic Criteria Step by step Approach

The diagnosis of Marfan syndrome (MFS) in familial and

sporadic cases are based upon the presence of characteristic manifestations, particularly aortic root dilatation/dissection and ectopia lentis, as well as other systemic features.

For patients with aortic aneurysm and/or dissection without a known mutation, aortic imaging is recommended for first degree relatives to identify those with asymptomatic disease.

DIAGNOSIS

Echocardiography is recommended at initial diagnosis and at six months to assess the aortic root and ascending aorta in patients with MFS.

Monitoring should be performed at least annually in patients with Ao root diameter more than 4.0 cm, and biannually in patients at higher risk (Ao root diameter more than 4.5 cm; Ao root enlargement more than 0.5 cm per year ; family history of Ao dissection).

AHA/ACC/STS 2010 recommendations

DIAGNOSIS

A Normal B MFS; Ao root dialation C Aorto Annular ectasia; whole Asc Ao dialation

MFS; Ao root dialation

DIAGNOSIS

MFS: Ao Regurgitation

DIAGNOSIS

DIAGNOSIS

DIAGNOSIS

DIAGNOSIS

Treatment varies on the affected organ. There is no known cure. Medical treatment and surgery can improve

patient’s long term outcome and their lifespan. Multidisciplinary approaches...

TREATMENT

Aortic root disease, leading to aneurysmal dilatation, aortic regurgitation, and dissection - main cause of morbidity and mortality.

TREATMENT

• It`s original introduction was by H Bentall and A De Bono in 1968. • Since then there have been several modifications to the procedure.

A composite graft replacement of the aortic valve, aortic root and ascending aorta, with re-implantation of the coronary arteries into the graft.

TREATMENT

• It`s original introduction was by H Bentall and A De Bono in 1968. • Since then there have been several modifications to the procedure.

TREATMENT

23 years young, Male Marfan`s Syndrome Palpitation, easy fatigability and chest

discomfort for 1 year NYHA FC III symptoms CXR: Dilatation of ascending aorta Echo: moderate AR, EF 48%, ascending

aortic aneurysm 5 cm in diameter, no evidence of ascending aortic dissection, sinus valsalva 6 cm, AV 3 leaflets rolling and retracted of leaflets, mild MR

CTA: Aortic aneurysm at ascending aorta size 5.5 cm in diameter.

National University Heart Centre, Singapore in January 2011

FOLLOW-UP OF A CASE

Clinical findings X-ray Chest Colour Dopplar Echocardiography Contrast-enhanced computed tomography Cardiac magnetic resonance images Cardiac catheterization with aortography

DIAGNOSTIC WORK-UP

Elective replacement of the aortic valve, aortic root and ascending aorta, with re-implantation of the coronary arteries by Composite valve graft with 27 mm St Judes Mechanical valved Conduit (Modified Bentall Procedure) on 20.1.2011

TREATMENT

•Femoral arterial Cannulation •Median Sternotomy

Modified Bentall Procedure

• Double Venous cannulation • Establish cardiopulmonary bypass

• Cross-clamping the aorta distal to the aneurysm

Modified Bentall Procedure

• Excise aortic cusps • Valve Sized

• Aorta cut open and gave Cardioplegia using Bainbridge (M) through the Left and Right Coronaries

Modified Bentall Procedure

• A composite valve graft with 27mm St. Judes Mechanical valved conduit was implanted.

Modified Bentall Procedure

•Subsequent reimplantation of the coronary arteries following open-button technique to the aortic graft.

Modified Bentall Procedure

• Haemostasis ensured

• Refashioning of the tube graft for distal anastomosis to the divided aorta.

Modified Bentall Procedure

• De-Airing • Gradual Weaning off CPB • De-cannulation

• Complete the distal anastomosis

Modified Bentall Procedure

• Postoperative course was

uneventful, and

• discharged well to home with

very good outcome.

• Being as Marfan`s Syndrome,

he was kept on regular follow up

in last 6 years.

• Medications-

• Warfarin

• Beta- Blocker

• Angiotensin-II Receptor Blocker

FOLLOW UP

He has been living a normal life activities with improved quality of life.

FOLLOW UP VISITS

What to look for ? Prosthetic valve function

MV

Ventricular function

Pseudoaneurysm/Anastomotic leak

Aneurysm / Dissection in remaining aorta

FOLLOW UP VISITS

At one year : CT Angio of whole aorta, Echo for valve and LV

Every year: Echo for valve and LV

If residual dissection or leak: CT Angio or MRI every year.

If no residual problem : MRI every five years

IMAGING PROTOCOL

RESULT

An average 86% long-term survival.

Depends largely on their preoperative condition.

Elective aortic root replacement have better outcomes than the procedure on an emergent basis.

An open-button technique is the appropriate choice for performing composite conduit replacement of the ascending aorta.

Typically, younger patients have better outcomes after aortic root replacement surgery than do older adult patients.

Most patients are able to fully resume their normal preoperative level of activity within six to eight weeks.

Cardiac rehabilitation program.

RESULT

Modified Bentall procedure provided satisfactory result in Marfans` syndrome

Type of surgery: depends on Patient condition

Age, comorbidity, condition of native aortic valve and aortic root, limitation of anticoagulant usage postop

Valve preference in each patient Surgeon (experience, skillful)

CONCLUSION

Because of the potential for late dissection or

aneurysm in other areas of the aorta, patients

with Marfan syndrome should have on regular

follow up with control of BP & serial computed

tomographic scans indefinitely.

A 23 years young male patient of Marfan`s Syndrome, was visited to National University Heart Centre, Singapore in January 2011 with the complaints of palpitation, easy fatigability and chest discomfort for last 1year with NYHA FC III symptoms. He was evaluated by echocardiography and CT angiogram which revealed he was having Ascending Aortic Aneurysm with the aortic root enlargement associated with moderate to severe aortic regurgitation & mild mitral regurgitation. He underwent Elective replacement of the aortic root by Composite valve graft with Mechanical valve (Bentall operation). We excised the aneurysm, implanted a composite graft with Mechanical prosthetic valve conduit (Modified Bentall operation), subsequent reimplantation of the coronary arteries to the aortic graft, and made the distal anastomosis to the divided aorta. Postoperative course was uneventful with very good outcome & in last 5 years regular follow up, he has been living with improved and normal life activities being as Marfan's syndrome.

Follow up of a case : Elective replacement of the aortic root in Marfan's syndrome by Composite valve graft with Mechanical valve (Bentall operation)

Correspondence:

Dr. Lt. Col. Md Abdul Hannan MBBS, FCPS (Surgery), FCPS (Cardiovascular Surgery),

Fellowship in CTVS (NUH, Singapore)

Classified Cardiovascular Surgeon CMH Dhaka, BANGLADESH

ABSTRACT

Lt Col MA Hannan1, Brig Gen Musa Khan2, Maj Gen Munshi MM Rahman3, Michael G. Caleb4 , C N LEE5

ABSTRACT: A 23 years young male patient of Marfan Syndrome, was visited to National University Heart Centre, Singapore in March 2011 with the complaints of palpitation, easy fatigability and chest discomfort for last 1year with NYHA FC III symptoms. He was evaluated by echocardiography and CT angiogram which revealed he was having Ascending Aortic Aneurysm with the aortic root enlargement associated with moderate to severe aortic regurgitation & mild mitral regurgitation. He underwent Elective replacement of the aortic root by Composite valve graft with Mechanical valve (Bentall operation). We excised the aneurysm, implanted a composite graft with Mechanical prosthetic valve conduit (Bentall operation), subsequent reimplantation of the coronary arteries to the aortic graft, and made the distal anastomosis to the divided aorta. Postoperative course was uneventful with very good outcome & in last 5 years regular follow up, he has been living with improved and normal life activities being as Marfan Syndrome.

Follow up of a case : Elective replacement of the aortic root in Marfan Syndrome by Composite valve graft with Mechanical valve (Bentall operation)

Dr. Lt. Col. Md Abdul Hannan, MBBS, FCPS (Surgery), FCPS (Cardiovascular Surgery), Fellowship in CTVS (NUH, Singapore), Classified Cardiovascular Surgeon, CMH Dhaka, BANGLADESH.