14. Revascularization of Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum and Right Ventricular-Dependent Coronary Circulation
Children's Healthcare of Atlanta
Atlanta, GA
United States - Contact Me
Paul Chai is Chief of Pediatric Cardiothoracic Surgery at Children's Healthcare of Atlanta/Emory University. He completed his general and cardiothoracic surgery residency at Duke University Medical Center, and his pediatric cardiothoracic surgical fellowship at Mott Children's Hosptial/University of Michigan.
Cleveland Clinic
Cleveland, OH
United States - Contact Me
Hani Najm, MD, is the Chair of Pediatric and Congenital Heart Surgery and Executive Co-Director of the Pediatric and Adult Congenital Heart Center at Cleveland Clinic. Dr. Najm is board-certified in surgery and cardiothoracic surgery.
Education and Training: Dr. Najm earned his medical degree from King Saud University, Riyadh, Saudi Arabia. He completed a rotating internship in Pediatrics, Obstetrics and Gynecology and a general surgery residency at King Khalid University. He also served a General Surgery residency at Ottawa Civic Hospital, Canada, and a Cardiothoracic Surgery residency at The Toronto Hospital and Hospital for Sick Children (SickKids), Canada. He completed fellowships in Pediatric and Adult Cardiac Surgery at Hospital for Sick Children (SickKids) and Sunnybrook Health Sciences Centre, Toronto.
He was appointed to the Cleveland Clinic staff in 2016 after serving as a consulting staff member in 2014. He has served at King Abdulaziz Cardiac Center in Saudi Arabia, including appointments as Deputy Chairman since 2008, Head of Cardiac Surgery since 2001, and Consultant for Adult and Pediatric Cardiac Surgery since 1999.
About Dr. Najm: I believe my greatest strength as a heart surgeon is my ability to offer the whole spectrum of heart surgery from the first day of life all through adulthood, including procedures that range from simple to the most complex, all with the best outcomes.
Leisure activities: In his leisure time, Dr. Najm enjoys Taekwondo. He is a 5th Dan black belt, earning 3 gold medals as the Saudi Arabia Champion.
Abstract
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.
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