We report here the management of a 57-year-old man who developed end-stage heart failure secondary to tricuspid atresia and ventriculoarterial concordance. Infants with tricuspid atresia commonly present with cyanosis due to decreased pulmonary blood flow, as in this case. Contemporary surgical management includes a systemic-to-pulmonary conduit, usually in the form of a modified Blalock-Taussig shunt, followed by creation of a Fontan circulation.1 Such patients have an excellent chance of reaching adulthood, as demonstrated by the SickKids group reporting 60% survival to 20 years with the Fontan procedure.2
In previous years, cyanosis was sometimes palliated with a Potts shunt (descending aorta to left pulmonary artery anastamosis),3 as was the case in our patient. This strategy has been abandoned because of difficulty in regulating pulmonary blood flow, with the risk for predisposing to pulmonary vascular disease and left ventricular overload. Our patient survived to adulthood but developed progressive congestive heart failure (stage D cardiomyopathy) complicated by atrial flutter. He had multiple failed cardioversions and was treated with amiodarone and warfarin.4 Cardiac magnetic resonance imaging demonstrated a patent shunt to the descending thoracic aorta with mild stenosis of the left pulmonary artery (Fig. 1, upper panel). The lungs were chronically exposed to systemic pressures as a result of the Potts shunt. Surprisingly, however, cardiac catheterization revealed normal pulmonary vasculature. The pressures were as follows: mean right pulmonary venous wedge pressure, 22 mm Hg; mean left pulmonary venous wedge pressure, 25 mm Hg; mean pulmonary artery pressure (PAP), 22 mm Hg; and pulmonary-to-systemic blood flow ratio, 2.3, with a pulmonary vascular resistance (PVR) of 1.5 Wood units. Other medical history is significant for chronic renal disease (baseline creatinine, 1.7 mg/dL), streptococcal viridans endocarditis, colon cancer with sigmoid colectomy and chemotherapy, and polycythemia secondary to heart failure.
Because PVR was normal, the patient was listed for heart transplantation but developed progressive dyspnea with worsening systolic and diastolic heart failure and was admitted to a hospital. During the admission, a heart became available. The patient underwent combined thoracic endovascular aortic repair (TEVAR) to eliminate the Potts shunt and subsequent orthotopic heart transplantation. Importantly, the Potts shunt had to be closed before cross-clamp and cardiac arrest because without it, the surgical field would be flooded with blood via the pulmonary artery. Initially, the chest was opened and the heart was exposed. The patient was heparinized, and aortic and bicaval cannulation were established. The femoral vessels were dissected, and a semirigid wire (Medtronic, Minneapolis, MN USA) was advanced from the right common femoral artery to the aortic root. Fluoroscopy revealed the Potts shunt at the junction of the proximal and the middle third of the descending thoracic aorta (Fig. 1, left lower panel). A Medtronic Valiant thoracic endovascular stent graft was deployed and modeled into place using a Reliant balloon (Medtronic, Inc, Minneapolis, MN USA) just distal to the left subclavian artery, completely occluding the Potts shunt (Fig. 1, right lower panel). There was an immediate decrease in the oxygen saturation, and the patient was then placed on cardiopulmonary bypass. There were no endoleaks, as confirmed using fluoroscopy. Orthotopic bicaval heart transplantation was performed in the usual manner.
Postoperatively, the patient required a moderate amount of inotropic support including epinephrine, norepinephrine, milrinone, isoproterenol, and vasopressin, which were weaned within 72 hours. The patient was extubated on postoperative day 2. However, while in the hospital, the patient developed progressive dyspnea and fatigue requiring reinstitution of milrinone, isoproternol, and sildenafil for right-sided heart failure as well as drainage of pericardial effusion on postoperative day 19. Inotropic support was slowly weaned, and the patient was discharged on day 30 on diuretic, sildenafil, and a standard immunosuppressive treatment regimen. At the time of discharge, cardiac catheterization demonstrated a PAP of 34 mm Hg/9 mm Hg and a PVR of 1.21 Wood units. Echocardiography demonstrated normal right ventricular (RV) function and RV systolic pressure of 9.9 mm Hg. The patient was readmitted within 24 hours for fever without any other symptoms. He was afebrile on admission, the chest was clear on auscultation, wounds were clean and dry, and white blood cell count was nonelevated (4600/μL). The findings from the chest x-ray were normal. He was pan cultured and placed on empiric vancomycin and ertapenem. Blood and urine cultures were negative for bacteria after 48 hours. The patient had no productive cough, so sputum culture was obtained. Antibiotics were discontinued after 72 hours, and he discharged home because there were no recurrence of his fevers and no other signs of infection.
At 3-month follow-up, cardiac catheterization revealed a PAP of 29 mm Hg/13 mm Hg and a PVR of 0.48 Wood units. Echocardiography demonstrated a dilated RV with normal systolic function and an RV systolic pressure of 24.7 mm Hg. At 1 month postoperatively, he had an incision and drainage of a groin lymphocele without event, and at 7 months, he had an upper respiratory tract infection treated with levofloxacin. At the last follow-up visit 7 months and 19 days postoperatively, the patient was free of heart failure symptoms and had returned to his daily activities.
Heart transplantation for complex congenital lesions and endovascular repair for Potts shunt have been reported separately, but to our knowledge, this is the first report of a combined TEVAR and heart transplantation for a patient with tricuspid atresia and a patent Potts shunt. Heart transplantation for complex congenital heart disease has been reported including that for tricuspid atresia, but the presence of a functional Potts shunt posed an additional surgical challenge.5
Surgical correction of the Potts shunt is complex and typically involves establishment of cardiopulmonary bypass and patch closure under deep hypothermic circulatory arrest. The posterior location of the shunt poses a surgical challenge, particularly in a redo setting, as in our patient. Transcatheter Potts shunt closure has been reported previously using the Amplatzer septal occluder (St. Jude Medical, Minneapolis, MN USA)6 and endovascular stent grafts (Zenith, Cook Medical, Bloomington, IN USA).7 Our approach uses an established endovascular technology, avoiding a high-risk redo dissection of the aorta through a left thoracotomy. Notably, because of the complexity of his anatomy and associated right-sided heart failure, he was not a candidate for left ventricular assist device support because he continued to deteriorate.
As mentioned, a significant risk associated with Potts shunts is creating a shunt that predisposes the patient to progressive pulmonary vascular disease and ventricular overload. Rarely, patients can live into adult life with a Potts shunt with a balanced pulmonary and systemic circulation.8 This was the case in our patient, but over time, he did develop the sequelae of uncorrected Potts shunt. Arrhythmias, as seen in our patient, are a common occurrence in patients with tricuspid atresia who survive to adulthood.
The primary indication for cardiac transplantation was the progression of congestive heart failure. A major consideration in this patient was to rule out increased PVR secondary to Potts shunt, which would not only predispose this patient to right-sided heart failure but, if present, would contraindicate a standard heart transplantation and require a combined heart/lung transplantation because of the increased PVR. Although our patient did develop some signs of right-sided heart failure in the immediate perioperative period, this was successfully treated with inotropic support and pulmonary vasodilators. Upon discharge and on follow-up assessment, right ventricular function and PAPs had normalized.
In this report, we demonstrate a patient who, to our understanding after review of the literature, is the oldest survivor with a Potts shunt for palliation of tricuspid atresia and discuss a successful combined TEVAR for shunt closure and heart transplantation procedure.
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