Repair of anomalous pulmonary artery with interposition graft: midterm results.
ABSTRACT Anomalous pulmonary artery arising from the aorta is a rare congenital anomaly. The midterm results of repair of this malformation by Gore-Tex graft interposition were examined in 5 patients: 3 with anomalous right pulmonary artery and 2 with anomalous left pulmonary artery from the ascending aorta. Echocardiography was adequate in 4 cases for diagnosis, planning the operation, and follow-up. Angiography was needed for diagnosis in one case where the echocardiographic findings were unclear. The mean follow-up period was 4 years. One patient with tracheoesophageal fistula and cardiac malformation died 2 months after the operation due to multi-organ failure. Three patients needed re-operation because of graft narrowing, and one was without problems 5.2 years postoperatively. In anomalous pulmonary artery from the ascending aorta, repair should be performed as early as possible to prevent pulmonary hypertensive changes. When the anomalous pulmonary artery cannot be anastomosed directly to the main pulmonary artery, an interposition graft can be placed safely without cardiopulmonary bypass. With appropriate follow-up, this can be a satisfactory solution, although it carries the risk of re-operation due to graft narrowing.
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ABSTRACT: Anomalous origin of the right pulmonary artery is a rare congenital malformation and is usually fatal without early surgical correction. A 16-year-old patient, who had received no previous treatment, demonstrated abnormal findings on ECG. On cardiac catheterization, the pressure in the normally arising left pulmonary artery was found to be greater than the systemic level. Bidirectional shunting across a patent ductus was identified and aortography revealed that the right pulmonary artery arose from the posterior wall of the proximal ascending aorta. The presence of severe pulmonary vascular obstructive disease was anticipated and right lung biopsy was performed. The index of pulmonary vascular disease rating was 2.8 and the Heath-Edwards classification was grade 3. Consequently, we considered that corrective right pulmonary circulation could be maintained after correction, and total correction was performed. The postoperative course was uneventful and the pulmonary artery pressure reduced significantly. Discussion focuses on the surgical indications for such infrequent older cases and we conclude that preoperative lung biopsy is useful in identifying severe pulmonary vascular disease and evaluating indications for surgery.The Japanese Journal of Thoracic and Cardiovascular Surgery 05/1998; 46(4):380-4. DOI:10.1007/BF03217759
- Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin 01/1970; 43(3):420-426. DOI:10.1007/BF01915623 · 2.56 Impact Factor
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ABSTRACT: A series of earlier reports has described the velo-cardio-facial syndrome (VCFS), a syndrome of multiple anomalies including cleft palate, heart malformations, facial characteristics, and learning disabilities. The patients reported previously were primarily ascertained from a craniofacial program at a large tertiary medical center. Recent reports, including a companion paper in this issue, suggest that this common syndrome of clefting is also a common syndrome of congenital heart defect (CHD) which is expressed as familial examples of DiGeorge sequence. Appreciation of more severely affected cases of VCFS and the detection of mild expressions have led to a broadening of the phenotypic spectrum of the syndrome. The purpose of this report is to describe the full spectrum of VCFS, including several new manifestations and to compare the VCFS phenotype with published cases of “familial DiGeorge sequence” which are now thought to represent examples of VCFS. © 1993 Wiley-Liss, Inc.American Journal of Medical Genetics 02/1993; 45(3):313 - 319. DOI:10.1002/ajmg.1320450307 · 3.23 Impact Factor