Revascularization of Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum and Right Ventricular-Dependent Coronary Circulation

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Case Video Submission 

Hani Najm (1), John Costello (2), Nicholas Oh (2), Miza Salim Hammoud (3), Munir Ahmad (1), Shahnawaz Amdani (2), Patcharapong Suntharos (2), Tara Karamlou (1), Chandrakant Patel (4)

(1) Cleveland Clinic, Cleveland, OH, (2) Cleveland Clinic Foundation, Cleveland, OH, (3) Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, OH, (4) Akron Children's Hospital, Akron, OH

*Hani Najm    -  Contact Me
Cleveland Clinic

John Costello    -  Contact Me
Cleveland Clinic Foundation

Nicholas Oh    -  Contact Me
Cleveland Clinic Foundation

Miza Salim Hammoud    -  Contact Me
Department of Pediatric Cardiac Surgery, Cleveland Clinic

Munir Ahmad    -  Contact Me
Cleveland Clinic

Shahnawaz Amdani    -  Contact Me
Cleveland Clinic Foundation

Patcharapong Suntharos    -  Contact Me
Cleveland Clinic Foundation

*Tara Karamlou    -  Contact Me
Cleveland Clinic

Chandrakant Patel    -  Contact Me
Akron Children's Hospital

*Hani Najm    -  Contact Me
Cleveland Clinic

Objective:
Pulmonary atresia with intact ventricular septum (PA/IVS) and right ventricular-dependent coronary circulation (RVDCC) presents unique challenges for survival due to the precarious coronary circulation. In this case we propose revascularization of the right ventricle with aortic oxygenated blood via a graft from the aorta to the hypoplastic tricuspid valve. This graft is in addition to a modified Blalock-Taussig-Thomas (mBTT) shunt to stabilize the newborn until the next procedure.

Case Video Summary:
This full-term, 4.4 kg, 3 week old neonate with PA/IVS and RVDCC was maintained on prostaglandin infusion following birth. With decrease in her pulmonary vascular resistance in her first couple of weeks of life, she had significant myocardial ischemia with ST segment changes and elevated troponin levels requiring intubation for stabilization. Heart transplantation was elected as a definitive pathway. To stabilize the precarious coronary circulation, a graft from the aorta to tricuspid valve was planned in addition to the mBTT shunt. After median sternotomy, a 3.5 mm Gore-Tex right mBTT shunt was placed between the innominate artery and right pulmonary artery off pump. A 5 mm saphenous vein homograft was sewn to the side of the ascending aorta. The distal arch and IVC were cannulated, cardiopulmonary bypass (CPB) initiated, the ductus ligated, and the heart immediately arrested in antegrade fashion to avoid myocardial ischemia given the coronary fistulas. Atrial septectomy was performed, and the saphenous vein homograft was routed into the right atrium (RA) through the RA wall and anastomosed directly to the 5 mm tricuspid valve annulus, thus intentionally making the tricuspid valve incompetent. After weaning from CPB successfully, the IVC cannula was exchanged for a Carmeda-coated cannula, and minimal left ventricular assist device (LVAD) support was initiated with PediMag at low flow of 70 ml/kg (300 ml) per minute. The patient was successfully weaned off LVAD support on postoperative day #3. She is currently still active (status 1Ae) on the transplant list.

Conclusions:
In PA/IVS with RVDCC, the use of an aortic to tricuspid graft delivers oxygenated blood to the right ventricle and avoids coronary ischemia and the need for long-term assist device support while either awaiting transplantation or the next stage in single ventricle palliation.

Single Ventricle Management