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Interact CardioVasc Thorac Surg 2007;6:820-822. doi:10.1510/icvts.2007.154625 © 2007 European Association of Cardio-Thoracic Surgery
Decision-making in unoperated adults with congenital heart disease: a difficult taskDivision of Cardiovascular and Thoracic Surgery, Department of Radiology, British Columbia Children's Hospital, Suite AB307, 4480 Oak Street, Vancouver, British Columbia V6H 3V4, Canada Received 20 March 2007; received in revised form 10 August 2007; accepted 14 August 2007
*Corresponding author. Tel.: +1 604 875-3165; fax: +1 604 875-3159.
The authors present a 32-year-old lady with a diagnosis of VSD, pulmonary atresia and multiple aortopulmonary collateral arteries (MAPCAs), who was palliated successfully by stent implantation. This interventional procedure is discussed as well as the decision-making process in adult patients with unoperated congenital heart disease.
Key Words: Catheter intervention; Adult congenital heart disease
Most patients with congenital heart disease will undergo intracardiac repair in early infancy and childhood. Despite early surgery and improved outcomes, many will require further operations, and will develop long-term complications [1]. There is a small group of patients with structural and/or valvular congenital heart disease who present late as adolescents or during adulthood [2]. As symptoms progress, some patients face the prospect of complex operations. Complete repair with its surgical risks needs to be weighed against a palliating procedure with lower risks that enables return to an acceptable level of activity. We present a patient with VSD, pulmonary atresia, and MAPCAs who was palliated by catheter intervention.
Our patient first presented at four years of age to a hospital in Taiwan with shortness of breath. She was born at term without complications, but had frequent respiratory tract infections during infancy. On exam, she was mildly clubbed with a hemoglobin of 18 g/l. Cardiac cath revealed pulmonary atresia, VSD, and MAPCAs. The surgical risk was deemed too high to proceed with complete repair. She was lost to follow-up until age 31 years when she became increasingly short of breath with exercise and was clinically more cyanotic. She denied chest pain, palpitations or syncope, with no previous surgery or major medical illness. She was working full time. Saturations on room air were 82%. Hemoglobin was 20.3 g/l. Chest X-ray showed normal heart size but decreased vascularity bilaterally. Echocardiogram showed normal AV valve connection, a large perimembranous VSD, and aortic override. The pulmonary artery could not be seen. CT and catheter angiography both identified small confluent native pulmonary arteries measuring 8 mm in diameter, and three large MAPCAs (Fig. 1a,b). The collateral to the left lower lobe was stenotic just beyond its origin. The left upper lobe MAPCA was also stenotic but appeared to have dual arterial supply, filling the left and right native pulmonary arteries in retrograde fashion. The MAPCA to the right lower lobe was quite tortuous and stenotic.
Bilateral unifocalization and complete repair was discussed but the patient refused surgery. Over the next two years, she became increasingly symptomatic. Her saturation was 80% at rest, dropping to the low 60s with exercise. Her hemoglobin was 22 g/l, with a hematocrit of 0.67. After discussion with the patient, we elected to stent the two left MAPCAs to improve her pulmonary blood flow as the risk of stent implantation was low vs. unifocalization and intracardiac repair. Under general anesthesia, right common femoral arterial access was obtained, with preliminary thoracic aortogram confirming two large left-sided MAPCAs and a single right-sided MAPCA, in addition to hypertrophied bronchial and intercostal arteries. The more superior of the two left-sided MAPCAs demonstrated continuity with the vestigial main pulmonary artery, with cross-filling into the right-sided pulmonary arterial tree. The other MAPCAs did not show dual supply to these lung segments. Systemic heparinization was done. The lower left-sided MAPCA was stented, using a Boston Scientific 8x29 mm Monorail Carotid Wallstent. The upper left-sided MAPCA was stented using 10x24 mm and 10x31 mm Carotid Wallstents, placed in tandem. Inflation pressures were up to 6 atmosphere for both dilation, and inflation time was 15 s for each site. The areas of stenoses were increased from 3 mm to 5 and 7 mm, respectively. Stent lengths were chosen to completely bypass the stenotic area. Post-stenting angiography confirmed markedly improved pulmonary blood flow to both lungs (Fig. 1c,d), with the patient's oxygen saturations rising to 90% on room air. The right common femoral arterial puncture site required cutdown and patch repair of the vessel. Post-procedure the patient's saturations dropped into the mid 80s with her chest X-ray demonstrating findings consistent with pulmonary edema. As she developed a cough with bloody sputum, a diagnosis of pulmonary reperfusion syndrome was made and managed conservatively. Her chest X-ray and clinical symptomatology resolved within 24 h (Fig. 2).
At follow-up, she is markedly improved and is maintaining oxygen saturations in the high 80s.
Patients who consider themselves asymptomatic nearly always have objective evidence of reduced exercise capacity. Deterioration can be due to increasing cyanosis, development of arrhythmias, ventricular and/or valvular dysfunction, or coronary artery disease. Patients with VSD and pulmonary atresia have the worst prognosis – survival without surgical repair beyond the fifth decade is exceptional [3, 4]. They may suffer from volume-loaded circulation, leading to heart failure and/or pulmonary hypertension. More than half will develop hemodynamically significant aortic valve regurgitation [3]. Patients with VSD and pulmonary atresia often have alternate sources of pulmonary blood flow through MAPCAs. As the pulmonary circulation can be extremely heterogeneous, it is important to define the pulmonary anatomy with precision as it determines the surgical approach. Particular attention should be paid to the presence, size, and branching of native pulmonary arteries, MAPCAs, and number of pulmonary parenchymal segments supplied by MAPCAs [5]. There are several features in performing reparative surgery in the adult, namely the systemic effects of chronic cyanosis, ventricular hypertrophy, abnormalities of ventricular function, and the frequency and importance of postoperative arrhythmias. Longstanding cyanosis creates difficulty in obtaining postoperative hemostasis, compounded by extensive suture lines and long bypass times [6–8]. Estimated long-term survival rates in repaired patients are approximately 92% at 4-year, 86% at 10-year, 80% at 15-year, and <75% at 20-year [7]. Adult patients with PA, VSD, and MAPCAs develop progressive cyanosis on a basis of hypertensive changes or collateral stenoses. There are the uncommon unoperated, or palliated patients who are unsuitable for complete repair, as was our patient. The use of endovascular self expandable stents become an attractive option [9]. Hybrid surgery may be considered to facilitate repair, although not entertained in our patient [10]. We therefore elected to stent both collaterals to the left lung as this would be technically easier than intervening on the very tortuous right lung collateral, and as the left superior MAPCA had dual supply. Her pulmonary reperfusion syndrome could be explained on the basis of the marked increase in pulmonary blood flow after the stenting procedures, into a pulmonary bed that was likely already maximally dilated in order to optimize flow and gas exchange in her chronically hypoperfused state. To minimize this syndrome, the stents were purposely not angioplastied, allowing self dilatation, and the patient was managed in an intensive care setting in order to treat the consequent pulmonary edema. Post-procedure, her oxygen saturation improved to the low 90s. She remains well palliated at follow-up with improved exercise tolerance. Decision-making regarding surgery in adult patients with congenital heart disease is often a difficult one. Patients may have a variety of symptoms, but are often quite functional. It is appealing to proceed toward a complete anatomical repair. However, if the decision is for late repair one needs to weigh the surgical risk and benefits against the potential risks for unoperated survival.
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Abstract
1. Introduction
