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At least 55 records · Page 3Linked to original sources
PubMed1999

Echocardiographic diagnosis alone for the complete repair of major congenital heart defects.

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.

Adolescent↗
PubMed1994

Surgical management of pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals.

Pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals is a form of complex congenital heart disease that is the subject of continued controversy. Disagreement exists regarding the appropriateness of surgical therapy, the timing of operation, and the optimal techniques for repair (1,2). The reason for these differences centers around the diversity of morphologic and consequent physiologic forms with which this defect presents. The relatively small number of patients and large number of subgroups make meaningful comparison between differing treatment strategies difficult. We present here the approach to these patients that is currently employed at UCLA Medical Center.

Abnormalities, Multiple↗
PubMed1997

Berry syndrome, a complex aortopulmonary malformation: one-stage repair in a neonate.

Successful one-stage repair of a Berry syndrome (interrupted aortic arch, distal aortopulmonary septal defect, right pulmonary artery branch originating from the ascending aorta, and intact ventricular septum) in the neonatal period has been reported in only 2 cases. We report the case of a newborn operated on with deep hypothermic arrest and isolated myocardial perfusion in whom the interrupted aortic arch was corrected by direct anastomosis between the ascending and descending aorta and the aortopulmonary septal defect was treated with reconstruction of the pulmonary trunk and right pulmonary artery, using a flap of aortic tissue. A native pericardial patch was used to reconstruct the ascending aorta.

Abnormalities, Multiple↗
PubMed2002

[Aortopulmonary window: clinical assessment and surgical results].

INTRODUCTION AND OBJECTIVES: Aortopulmonary septal defect is an uncommon congenital cardiac anomaly. To date, approximately 300 cases have been reported. We present our experience, emphasizing the importance of early correction to avoid irreversible pulmonary hypertension. PATIENTS AND METHOD: Between 1979 and 2000, seven patients underwent surgical repair of this heart defect in our hospital. Two had type I (proximal), 4 had type II (distal) and 1 had type III (complete). Complex associated cardiac anomalies were present in 4 cases: type A interruption of the aortic arch in 2 cases, hypoplastic aortic arch in 1 and transposition of great arteries with ventricular septal defect in 1. Four cases (57%) were diagnosed by echocardiography. In all patients diagnoses were confirmed by cardiac catheterization. Patient records were reviewed retrospectively, with special attention to clinical, echocardiographic and hemodynamic data as well as surgical characteristics. RESULTS: No intraoperative deaths occurred. The patient with associated transposition of great arteries died 22 days after surgery as a result of severe pulmonary hypertension. The remaining patients are asymptomatic without treatment after a mean follow-up period of 69 months. CONCLUSIONS: Even though aortopulmonary septal defect is a rare anomaly, it should be considered whenever the course of complex congenital heart disease includes early cardiac failure and pulmonary hypertension. Repair before 6 months will prevent irreversible damage of pulmonary vessels.

Abnormalities, Multiple↗
PubMed1992

New surgical technique for total-defect aortopulmonary window.

A new technique is described to repair aortopulmonary window with total defect in an 8-day-old baby. Because we expected the future growth of aorta, we used the anterior wall of the pulmonary artery as a large flap to reconstruct the posterolateral aortic wall. An equine pericardial patch was used to repair the defect in the pulmonary artery. This is a logically effective method for aortic reconstruction in a neonate with a large aortopulmonary septal defect.

Aortopulmonary Septal Defect↗
PubMed1999

As originally published in 1992: new surgical technique for total-defect aortopulmonary window. Updated in 1999.

A new technique is described to repair aortopulmonary window with total defect in an 8-day-old baby. Because we expected the future growth of aorta, we used the anterior wall of the pulmonary artery as a large flap to reconstruct the posterolateral aortic wall. An equine pericardial patch was used to repair the defect in the pulmonary artery. This is a logically effective method for aortic reconstruction in a neonate with a large aortopulmonary septal defect.

Aortopulmonary Septal Defect↗
PubMed1995

Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.

Traditionally patients with pulmonary atresia, ventricular septal defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. We have taken a different approach to this lesion. Between August 1992 and March 1994, ten patients aged 1.43 months to 37.34 years (median 2.08 years) at the severe end of the morphologic spectrum of this lesion underwent a one-stage complete unifocalization and repair from a midline sternotomy approach. The median Nakata index of true pulmonary arteries was 50.0 (range 0 to 103.13) and they provided vascular supply to up to nine lung segments (median 5 segments). The number of collaterals per patient ranged from two to five with a median of four. The collaterals provided vascular supply to a median of 15 lung segments per patient (range 11 to 20). Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. In only one patient (37.34 years old) was it necessary to use a non-native conduit for peripheral pulmonary artery reconstruction. The ventricular septal defect was left open in one patient (5 years old) because of diffuse distal hypoplasia and stenosis of the pulmonary arteries and the collaterals. The postrepair peak systolic right ventricular/left ventricular pressure ratio ranged from 0.31 to 0.58 (median 0.47). There were no early deaths. Complications were bleeding necessitating reexploration in one patient, phrenic nerve palsy in three patients, and severe bronchospasm in three patients. Follow-up (median 8 months, range 2 to 19 months) was complete in all patients. One patient was reoperated on for pseudoaneurysm of the central homograft conduit and then again for stenosis of the left lower lobe collateral. After this last operation at 13 months after the initial repair she died of a preventable cardiac arrest caused by pneumothorax. The patient with open ventricular septal defect underwent balloon dilation of the unifocalized pulmonary arteries, with a current pulmonary/systemic flow ratio of 1.4 to 1.8:1, and is awaiting ventricular septal defect closure. One other patient underwent balloon dilation of the reconstructed right pulmonary artery, with a good result. All survivors (9/10) are clinically doing well. This approach establishes normal cardiovascular physiology early in life, eliminates the need for multiple systemic-pulmonary artery shunts and use of prosthetic material, and minimizes the number of operations required.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed2004

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach.

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels. METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications. CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.

Abnormalities, Multiple↗
PubMedDate not supplied

One-stage repair of interrupted aortic arch and aortopulmonary window with an autologous arterial flap.

Interrupted aortic arch associated with an aortopulmonary window is a rare congenital malformation that requires an early diagnosis and surgical treatment to avoid irreversible pulmonary vascular changes. We describe herein successful one-stage repair in a neonate without the use of pericardium or other prosthetic material. Use of a transaortic approach permitted both accurate planning of the appropriate size of pulmonary trunk flap and also facilitated easy repair of the aortopulmonary septal defect. The growth potential of both great arteries was optimized.

Anastomosis, Surgical↗
PubMed1998

Monosomy 22q11 in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

OBJECTIVE: To describe the morphology of the pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries with and without monosomy 22q11. DESIGN: A retrospective analysis of all patients with this congenital heart defect who are being followed at the University Children's Hospital Erlangen. SETTING: A tertiary referral centre for paediatric cardiology and paediatric cardiac surgery. PATIENTS: 21 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Monosomy 22q11 was diagnosed by fluorescent in situ hybridisation using the D22S75 probe (Oncor). The morphology of the pulmonary arteries was assessed on the basis of selective angiograms. RESULTS: 10 patients (48%) were shown to have a microdeletion in 22q11 (group I). There was no difference with respect to the presence of confluent central pulmonary arteries between these patients (80%) and the remaining 11 patients (group II) without monosomy 22q11 (91%). Patients of group I, however, more often had arborisation anomalies of the pulmonary vascular bed (90% in group I v 27% in group II). Because of the more severe abnormalities of the pulmonary arteries, a biventricular repair had not been possible in any of the children with monosomy 22q11, though repair had been carried out in 64% of the children in group II. CONCLUSION: The developmental disturbance caused by the monosomy 22q11 seems to impair the connection of the peripheral pulmonary artery segments to the central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, resulting in a lower probability of biventricular repair.

Adolescent↗
PubMed1992

Detection of aortopulmonary window with ventricular septal defect by Doppler color flow imaging.

Aortopulmonary window is a rare congenital cardiac anomaly. When it coexists with a ventricular septal defect, the accurate diagnosis of aortopulmonary window on the basis of clinical examination is difficult. We report the case of an infant who had an aortopulmonary window together with a ventricular septal defect. An accurate diagnosis could be attained by visualization of the defect using two-dimensional echocardiography and detection of the flow through it by Doppler color flow imaging.

Aortopulmonary Septal Defect↗
PubMed2004

[Berry syndrome: successful neonatal repair of a rare complex cardiac malformation].

Aortopulmonary septal defect, interrupted aortic arch, aortic origin of the right pulmonary artery, intact ventricular septum, and patent ductus arteriosus (Berry Syndrome) is a rarely reported malformation. A 6-day-old girl underwent successful one-stage complete repair of direct anastomosis between the ascending and descending aorta, reimplantation of the right pulmonary artery augmented with pericardium patch. Stenosis of the origin of the reimplanted right pulmonary artery was treated by balloon angioplasty at the age of 3 months. The authors emphasize the additive nature of surgical correction and interventional cardiology. Review of the literature reveals this patient to be the 25th reported case with Berry syndrome, and the first of that in Hungary.

Angiography↗