Echocardiographic diagnosis alone for the complete repair of major congenital heart Defects

Abstract Objectives. The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart Defects undergoing primary complete repair. Background. Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex Defects. Methods. To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart Defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular Septal Defect, truncus arteriosus, Aortopulmonary Septal Defect, and totally anomalous pulmonary venous return. We excluded children with less complex Defects such as isolated shunt lesions, as well as those with the most complex Defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery. Results. Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality. Conclusions. This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart Defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.

early and late results after repair of Aortopulmonary Septal Defect and associated anomalies in infants 6 months of age

Abstract 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.

Early and Late Results After Repair of Aortopulmonary Septal Defect and Associated Anomalies in Infants < 6 Months of Age

Abstract 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.

prenatal diagnosis of Aortopulmonary window with interrupted aortic arch by fetal echocardiography

We report the first case of prenatally diagnosed Aortopulmonary window (APW) with interrupted aortic arch. The mother was referred for fetal echocardiography at 29 weeks’ gestation because of suspected congenital heart disease. On the short-axis view, we clearly imaged the Aortopulmonary Septal Defect at the proximal pulmonary artery and type A interrupted aortic arch. The window was 4 mm and the shunt flow detected by color flow Doppler was right-to-left in systole and early diastole. All aortic arch branches originated from the aorta proximal to the interruption. The well-developed ascending aorta was not continuous with the descending aorta in the sagittal view of the aortic arch. Visualization of the direction of the shunt flow across the APW and each origin of subclavian arteries was useful for the diagnosis of APW with interrupted aortic arch.

surgical experience for the treatment of Aortopulmonary Septal Defect

Objective Aortopulmonary Septal Defect is rare and accounted for abont 0.15% to0.60% of infants and children with congenital heart malformation. Aortopulmonary Septal Defect has similar pathophysiologic findings with patent ductus. Early pul-monary hypertension and congestive heart failure may develop as a result of large left to right shunt from the ascending aorta to the pul-monary artery, with a relatively rapid progression of pathological changes in the pulmonary vessels associated with pulmonary hyperten-sion. This article will describe our surgical experience in the treatment of this disease. Methods From December 1987 to March 2000,16 cases with Aortopulmonary Septal Defect underwent surgical treatment, 11 were males and 5 were females. The mean age and weight were (8.2 ± 7.1) years (range from 5 months to 22 years) and (22.2± 13.9) kg (range from 5.8 to 50.0) respectively. Seven cases were type Ⅰ, seven were type Ⅱ and two were type Ⅲ according to Richardson’s classification. The mean scale of aortop-ulmonary Septal Defect was (22.6± 7.3)mm (range from 10 to 35) . Coexistent ventriculor Septal Defect was found in two cases, atri-al Septal Defect was found in another two cases, patent ductus in one case and interrupted aortic arch (type B) in two cases. Cardiac catheterization were perforomed in ten cases, and the systolic and mean pulmonary artery pressure were (75.4± 25.2) mmHg (range from 39 to 120) and (55.1± 25.2) mmHg (range from 21 to81) respectively. All patients underrwent surgical repair under cardiop-ulmonary bypass (CBP) at moderate hypothermic temperature. Deep hypothermic circulatory arrest was used in two cases with inter-rupted aortic arch. The mean duration of aortic clamping and CPB were (70.4± 48.1) minutes and (110.5± 62.9)minutes. Strate-gies for APW repair were trans-aortic with patch closure in thirteen cases, trans-pulmonary in two cases and trans-window in one case.Other coexistent malformations were corrected simultaneously. Results The poet-operative systolic and mean pulmonary artery pres-sure were decreased to (50.2±18.5) mmHg (range from 20 to 78 mmHg) and (40.0± 15.2) mmHg (range from 18 to 65 mmHg) respectively. The mean duration of postoperative mechanical ventilation was (22.6 ±14.5) hours (range 12-70), that for ICU-staying was (45.8 ± 55.5) days (1-70), and that for hospitalization was (49.1 ±32.2) days (14-127). Two APW cases with interrupted aortic arch died of lethal acute renal failure and low cardiac output syndrome perioperatively. During the mean follow-up period of (8.8±7.5) years, there was no re-operation case or late death in 14 survived patients. All of them showed improvement in the heart function except for one patient who had small residual shunt. Pationts who recovered have gone to work or to school with the resolution of their murmus and significant improvement in the pulmonary congestion. Their enlarged hearts before operation have reduced by var-ious extents. Condusion Given the early pathological changes of pulmonary vessels in patients with Aortopulmonary Septal Defect,early diagnosis and surgical treatment, which may decrease the mortality, are of critically important. Coexistent and complicated con-genital Defects such as interrupted aortic arch may increase the mortality in APW cases.

Key words:
Aortopulmonary Septal Defect; Cardiac surgical procelures