Heart-Hand Syndrome in a Young Adult Filipino

By Leica Mariz Balanon Royeca
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Leica Mariz Balanon Royeca


Leica Mariz Balanon Royeca1


Philippine Heart Center, Philippines1
Structural Heart Disease - Congenital Heart Disease (ASD, PDA, VSD)

Heart-Hand Syndrome in a Young Adult Filipino

Leica Mariz Balanon Royeca1

Philippine Heart Center, Philippines1

Clinical Information

Patient initials or Identifier Number


Relevant Clinical History and Physical Exam

N.A. is a 25 years old male, Filipino who was acyanotic at birth.  At 1 year old, he had pneumonia and a murmur was noted. 2ded showed PDA and bicuspid aortic valve. Surgical correction was advised but lost to follow-up.1 week prior to consult, his easy fatigability worsened. On admission, he was acyanotic. There was no differential BP.  PMI was at 6th ICS midaxillary line with LV heave, regular rhythm and gr 3/6 systolic murmur at the 2nd ICS left parasternal line radiating to the carotids.

Relevant Test Results Prior to Catheterization

Hand xray: brachymetacarpia of the 2nd, 4th and 5th digits left
Pre op 2decho:-       LVEDD 9.4 LVESD 7.9 EF 36 RVFAC 49 TAPSE 2.2-       CHD, PDA (1.12cm) with predominantly left to right shunt (Qp:Qs 2.6:1)-       Bicuspid Aortic Valve with moderate AS, AVA 1.3cm2 by continuity equation, peak velocity 3.7m/s-       Moderate AR-       Mild MR and Trivial  TR-       Eccentric LVH with global hypokinesiaNormal Pulmonary artery pressure (SPAP 29mmHg)

Relevant Catheterization Findings

Hemodynamic Studies and Coronary angiogram:-       Angiographically normal coronary arteries-       Congenital Heart Disease-       Patent Ductus Arteriosus with left to right shunt Qp:Qs 4.18:1-       Bicuspid Aortic Valve with Moderate AS with systolic pressure gradient of 27mmHg and mild aortic regurgitation, Seller’s 1-       Mild pulmonary hypertension, MPAP of 56mmHgRP/RS of 0.04

Interventional Management

Procedural Step

Patient was placed on supine position under GETA. Pre-operative transesophageal echocardiogram revealed a bicuspid aortic valve with fused noncoronary and right coronary cups. Mitral valve annulus was dilated at 5.3cm.  Mid-line sternotomy done. Pericardium was opened and deflected.  Aortic and bi-caval cannulation done. Left ventricular vent and antegrade plegia-line were placed.  Cardiopulmonary bypass was initiated. Cross clamp applied and cardioplegia delivered.  PDA dissected and isolated, then ligated proximally and distally.  Posterior left atrial wall was opened. Mitral valve was inspected, annular sutures were placed,  and annuloplasty ring was implanted.  Left atrium was closed, aortotomy was done.  Aortic valve was examined and resected, annular sutures were placed mechanical aortic valve was implanted.  Aortotomy was closed. patient was rewarmed and weaned of bypass. Decannulation was done.  JP drains placed in right pleura, left an right mediastinum. CTT was placed at anterior mediastinum.  Anterior chest was closed in layers.  Post-operative TEE showed a normally functioning mechanical aortic valve with no paravalvular leak.  Residual shunt across the PDA was noted.

Case Summary

Post-operative recovery was tolerated and uneventful.  Patient was discharged with the following medications: Warfaring 5mg 1/2 tablet once daily, Sildenafil 25mg 1 tablet twice daily, Furosemide 40mg 1/2 tablet once daily and Spironolactone 50mg 1 tablet once daily. Upon follow-up at the outpatient department, patient reported improvement in functional capacity.
Franklin Espinoza Mori (ESSALUD) Aug 12, 2022
Excellent presentation