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Aortopulmonary septal defect and tetralogy of Fallot is a rare combination. We report a case of tetralogy of Fallot, non-confluent pulmonary arteries with the left arising from the arterial duct, and a large aortopulmonary septal defect diagnosed by echocardiogram and confirmed by cardiac catheterization.
Three patients are described in whom an aortopulmonary septal defect (aortopulmonary window) coexisted with a ventricular septal defect and pulmonary atresia. One patient had mild and another, moderate aortic regurgitation. In addition, one patient had a sinus of Valsalva aortic aneurysm, while another had a single coronary artery arising from the pulmonary trunk. One patient underwent surgical correction in infancy; the other two, in early adult life. In all three patients, surgical correction involved closure of the aortopulmonary window, closure of the ventricular septal defect, and placement of a valved conduit between the right ventricle and the distal pulmonary trunk. One patient died 3 weeks postoperatively due to secondary hemorrhage where the conduit had been sutured to the right ventricle. The other two patients are alive and well 3 1/2 years and 6 months after surgery, respectively. The presence of aortopulmonary window permits normal development of the pulmonary arteries in the presence of the coexisting pulmonary atresia; in the three patients described, the absence of pulmonary vascular disease made total surgical repair feasible for this combination of defects.
Surgical repair of an aortopulmonary septal defect, interrupted aortic arch and the duct that coexists in this setting usually includes closure of the aortopulmonary septal defect, reconstruction of the arch, and interruption of the duct. A 20-month-old girl underwent a successful one-stage repair of such anomalies in which an intraarterial baffle was used. A Gore-Tex baffle was used both to close the aortopulmonary septal defect and to conduct the ascending aortic blood flow through the duct into the descending aorta. We recommend use of this intraarterial baffle as an alternative to the repair of an aortopulmonary septal defect and an interrupted aortic arch with a widely patent duct.
OBJECTIVE: To study the surgical treatment of aortopulmonary septal defect. METHOD: From January 1983 to December 1996, 5 patients with aortopulmonary septal defect (APSD) (2 male and 3 female) aged from 6 to 34 years (mean 17.4 years) underwent surgical treatment. According to Mori's were classification, 5 patients belonged to type I, 2 type II and 1 type III. The diameters of the defect ranged from 1.0 to 3.0 cm. The operations were performed under moderate hypothermic cardiopulmonary bypass. Pulmonary artery incision was performed, in 3 patients while aortic incision in the other 2. All defects were repaired by Dacron patch. RESULT: There were no operative deaths and postoperative complications. During follow-up of 10 months to 12 years, no late complications and deaths were found. CONCLUSION: The operation should be done early as possible whenever the diagnosis is established. The surgical repair should be taken under cardiopulmonary bypass. To chose an incision on the aorta is more reasonable than on the pulmonary artery. The use the dacron patch can prevent stenosis and recanalization after operation.
Twenty-five patients with malseptation of the aortopulmonary trunk resulting in aortopulmonary septal defect (window) were evaluated in a unique combined surgical series assembled from two institutions participating in the USA-USSR Health Exchange Program. Typical aortopulmonary septal defect or window (type I) with the connection between the ascending aorta and main pulmonary artery was found in 21 patients. Four had a more cephalad defect (type II) between the ascending aorta and the origin of the right pulmonary artery. Hemodynamics were the consequence of a large left-to-right shunt (mean ratio of pulmonary to systemic flow, 3.0) with right ventricular hypertension (mean right ventricular pressure, 86 mm Hg) and increased pulmonary vascular resistance (mean, 7.4 U) (mean ratio of pulmonary to systemic vascular resistance, 0.33). Angiography may provide patterns that allow preoperative distinction between the two types of aortopulmonary septal defect and provide information important in planning the details of corrective operation. Operative techniques included ligation, direct suture, and patch closure of the aortopulmonary septal defect. The best method appeared to be patch closure by the transaortic approach; real and potential problems were associated with other techniques.
A new technique is described for repairing distal aortopulmonary septal defect with aortic origin of the right pulmonary artery in a 26-day-old neonate. To prevent the narrowing of the proximal right pulmonary artery in the distal aortopulmonary septal defect caused by conventional intraluminal prosthetic patch reconstruction, rerouting of the right pulmonary artery using native aortic wall tissue without artificial material was performed. This method seems to be superior to the conventional method, especially during the neonatal period.
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Case history and necropsy findings of a 5-month-old infant with a unique heart defect with features of truncus arteriosus communis and aortopulmonary defect in combination with severe tricuspid stenosis are presented. There is a wide spectrum of remarkable heart defects between truncus arteriosus communis and aortopulmonary septal defect.
In a 20-days old girl, aortopulmonary septal defect (total defect type) was closed by transaortic patch repair with cardiopulmonary bypass (CPB). The postoperative course was uneventful, and she was discharged 43 days later. This is the youngest and lowest-weight case ever reported in Japan. Various procedures for aortopulmonary septal defect have been proposed with or without CPB. However, transaortic repair with the aid of profoundly hypothermic circulatory arrest seems to be the most preferable corrective surgery for total defect type of neonate and low-weight infant.
A patient with a large congenital aortopulmonary septal defect lived for 46 years. Such lesions are usually fatal in early life unless corrected. Because the anatomic and physiologic adjustments were evident and relatively stable, the case calls for examination of the practice of prognostication.
Aortopulmonary septal defect, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the aorta comprised a challenging constellation of defects in a 13-day-old neonate, who underwent complete repair without homograft tissue or synthetic graft. After the aorta was separated from the pulmonary artery, the descending aorta was anastomosed to the septal defect in the ascending aorta. The right pulmonary artery was anastomosed to the septal defect in the main pulmonary artery, anterior to the aorta. The postoperative course was uncomplicated, and the potential for growth of both great vessels was optimized.
The association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum and interruption or coarctation of the aortic isthmus has not previously been reported as a syndrome. This combination of anomalies was encountered in five new patients and was previously reported in three. Two patients have undergone surgery with successful results. In contrast to the sagittally oriented conventional proximal aortopulmonary septal defect, the patients in this series had a more distal type of defect, possibly representing a partial persistence of the common arterial trunk. The pulmonary arterial bifurcation may malattach to this undivided truncal segment and, as a result, the right pulmonary artery may be partially or completely shifted into the aorta. This abnormal right pulmonary arterial origin may lead to "steal" from the aortic flow during embryogenesis and to hypoplasia of the aortic arch. This concept is supported by the angiographic observation that the greater the rightward displacement of the right pulmonary artery, the greater the hypoplasia of the arch. The diagnostic angiographic sign is a strikingly high origin of the right pulmonary artery together with aortic arch hypoplasia or atresia. Closure of the aortopulmonary septal defect with implantation of the right pulmonary artery in the pulmonary trunk and repair of the aortic arch anomaly is the recommended surgical treatment.
The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.
From 1981 to 1992, 10 infants with aortopulmonary septal defect (APSD) underwent surgical repair. The mean age at operation was 5.6 +/- 5.5 months, and the mean weight 4.6 +/- 2 kg. Intracardiac associated anomalies were as follows: ventricular septal defects (7 cases), tetralogy of Fallot (2 cases), aortic valve stenosis (2 cases), atrial septal defect (3 cases), patent ductus arteriosus (3 cases), pulmonary valve stenosis (1 case). Cardiac catheterization was performed in 8 out of 10 patients. Eight patients had type I (proximal) defect, 1 had type II (distal) defect and 1 had type III (absent aortopulmonary septation) defect. A variety of surgical procedure was employed. APSD closure with hemoclip was feasible in 3 cases with small window. In 6 patients, during a period of cardiopulmonary by-pass, a side biting clamp was positioned on the ascending aorta close the defect; the border of the window was divided leaving a flap of pulmonary wall on the left side to close the aortic defect; the pulmonary artery was repaired by an autologous pericardial patch. In the patient with type III APSD, aortopulmonary septation was carried out through a transwindow approach. Associated anomalies were repaired in all infants except one. Hospital mortality was 10% (1 case). No late deaths occurred. At a mean follow-up of 47 +/- 35 months 8 patients are asymptomatic and 1 is awaiting for repair of associated anomalies. Conclusions. APSD is a rare but nonetheless well identifiable anomaly. Surgery is indicated as soon as the diagnosis is established, regardless of the patient's age.(ABSTRACT TRUNCATED AT 250 WORDS)
Fourteen patients with malseptation of the aortopulmonary trunk underwent operative repair from 1978 to 1988. Age ranged from 1 year to 35 years (mean age, 9.6 years). Five patients had type I, 6 had type II, and 3 had type III lesions. The hemodynamic disturbance in all patients was the consequence of a large left-to-right shunt (mean pulmonary/systemic flow ratio, 2.38:1) with increased pulmonary vascular resistance (mean value, 4.47 units/m2). Our initial surgical experience with closure under cardiopulmonary bypass through the transaortic route in 3 patients and the transpulmonary approach in two patients resulted in 3 deaths. In the 9 subsequent patients, division and repair of the defect in the great vessels yielded uniformly good results. During follow-up, which ranged from 3 months to 2 years, all 11 survivors had good clinical improvement and none showed residual defects on restudy. Pulmonary artery pressure and pulmonary vascular resistance decreased in all patients except 1.
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