Tetralogy of fallot with pulmonary atresia and a major aortopulmonary collateral artery (MAPCA) in a young adult: A case report

CLINICAL PRESENTATION: This report describes a case of a 24-year-old, acyanotic, female patient presenting with mild effort dyspnea, a right ventricular heave, a displaced cardiac apex, a loud single S2, a holosystolic murmur on the left parasternal area, and a continuous murmur at the left posterior chest. Transthoracic and transesophageal echocardiography demonstrated dilated left atrium and ventricle, hypertrophied right ventricle and a large ventricular septal defect with overriding of the aorta. However, both studies failed to visualize the pulmonic valve. The patient underwent cardiac catheterization which revealed the presence of pulmonary atresia. A descending aortogram showed the presence of a single large major aortopulmonary collateral artery (MAPCA) arising from the descending thoracic aor ta which supplies confluent left and right pulmonary arteries. The pulmonary arterial vasculature is enlarged and pruned in appearance. The main pulmonary artery is non-atretic. Mean proximal pulmonary artery pressure was 68 mmHg, indicating severe pulmonary hypertension. Patient wasstarted on digoxin and sildenafil.SIGNIFICANCE: This case report emphasizes the importance of a well-developed central pulmonary circulation and an adequate but not excessive pulmonary blood supply from an aortopulmonary vascular connection in the survival of patients with pulmonary atresia into adulthood.

Ventricular Septal Defect and Pulmonary Atresia with Major Aorto-Pulmonary Collateral Artery: Diagnosis, Management and Review

PURPOSE: Based on our previous studies on ventricular septal defect (VSD), pulmonary atresia (PA) and major aortopulmonary collateral arteries (MAPCA), a management strategy formulated. To evaluate this strategy, it applied to a selected group of patients and outcome measured. METHODS: A management strategy was to promote growth of pulmonary arterial confluence (PAC), initially by right ventricle-pulmonary artery (RV-PA) conduit operation, later by balloon and or surgical angioplasty. Unifocalization performed only after PAC became big enough. If PAC absent, management plan discussed with surgeons after complete work-up. This strategy applied to four consecutive patients, initially diagnosed to have VSD+PA with MAPCA from January 2000 to December 2000. RESULTS: The age at diagnosis ranged from 8 days-2.5 months. PAC present in 3 children, all of whom underwent initial RV-PA conduit operation at the age range of 15 days-5 months. The remaining one patient not have PAC, underwent bilateral unifocalization at the age of 4 months. All had multiple operations (2.5 operations in average) and multiple interventions (4.5 interventional catheterizations in average) before reached to corrective or semi-corrective operation. All of them currently alive and have had either corrective operation (2 patients) or semi-corrective operation (fenestration in VSD patch in 2 patients). All but one asymptomatic and on no medication. CONCLUSION: Our management strategy for VSD+PA with MAPCA seems to work well. The cases with increased pulmonary blood flow needs meticulous plan to prevent both pulmonary vascular obstructive disease and naturally occurring pulmonary arterial stenosis and obstruction. However further study with more patients necessary.

Diagnostic Assessment of Pulmonary Atresia with Ventricular Septal Defect; Comparison of Echocardiogram with Cardiac Angiography

PURPOSE: Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. METHODS: This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations including echocardiography were done, catheterization/angiography was performed the next day and the results were compared with those of echocardiography. The study population consisted of 9 patients, diagnosed as PAVSD in our hospital, from Jan. 1995 to Dec. 1997. RESULTS: Pulmonary blood was supplied via ductus arteriosus, in 3 patients(group 1), and in the other 6 patients, via major aortopulmonary collateral arteries(MAPCA)(group 2). The characteristic findings of group 2 were cardiac murmur heard in the back area(3 cases), and abnormal pulmonary arborization pattern(2 cases). The echocardiographic diagnosis of source of pulmonary blood flow was correct in all cases except one case-who had had two previous shunt surgeries and additional MAPCAs. In group 2, the presence of pulmonary confluence was predicted in 3 out of 4 cases. The mean number of MAPCAs found by echocardiography was 2.3+/-1.2 and by angiography, 3.5+/-1.4. CONCLUSION: In evaluation of PAVSD patients, near complete characteristics of pulmonary blood supply were identified by echocardiogram. Using it, practitioners can make decisions for shunt surgery or the need for further evaluations, including cardiac catheterization which can be more easily executed with previous echocardiographic data.

Origins, Distributions and Characteristics of Collateral Circulation in Pulmonary Atresia with Ventricular Septal Defect: A Cineangiographic Study

BACKGROUND: The sources of pulmonary blood flow in patients with pulmonary atresia and ventricular septal defect (PA/VSD) are very diverse, and detailed informations for the pulmonary circulation are essential for successful treatment. The purpose of this study is to examine and define the origins, distributions and characteristics of collateral circulations in patients with PA/VSD and major aortopulmonary collateral arteries (MAPCAs). METHODS: 34 patients diagnosed as PA/VSD and MAPCA were studied between May 1992 and April 1996. 85 MAPCAs were investigated for the sites of origin, distributions and characteristics by review of biplane angiograms. The characteristics of MAPCAs were examined in the aspects of anastomosis sites and sites/types of stenosis. RESULTS: 1) In 34 subjects, a total of 85 MAPCAs existed which supplied blood to 344 bronchopulmonary segments (4.1 BPS/MAPCA). 2) The origin sites of MAPCAs were the descending aorta (72%), the branch of aortic arch (16%), and the aortic arch or ascending aorta (12%). 3) The BPSs which frequently take primary blood supply from MAPCA were the 3 BPSs of upper lobe and superior / posterior basal segment of lower lobe in the right lung, and the superior / posterior basal segment of lower lobe in the left lung. 4) The BPS having apparent dual blood supply was 13.4% and the most frequent was the superior and posterior basal segment of the right lower lobe. 5) In total, 64% of MAPCA had anastomosis, and anastomosis in the lobar pulmonary artery was 48% to be the most frequent. 6) Stenosis was observed in 61% of MAPCAs. The site of stenosis was midportion (33%), originating portion (27%), multiple (26%), peripheral (14%), and the type of stenosis was focal (53%), segmental (31%), diffuse (16%), in the order of frequency. CONCLUSION: In PA/VSD and MAPCA, selective angiogram should be performed in all the different blood-supplying sources of the lung for detailed information of the pulmonary circulation. When the blood sources of the pulmonary circulation is uncertain, elaborated efforts to find the blood sources, by angiograms in the frequent locations of MAPCA, are neccessary. We believe this study to be useful for precise planning in the diagnosis and treatment of PA/VSD and MAPCA.