Managing Ventricular Septal Defect with Associated Aortic Regurgitation: Two Decades of Experience.

BACKGROUND AND AIM OF THE STUDY: Ventricular septal defect (VSD) with aortic regurgitation (AR) is a well-known association. However, there is still no agreement about its management, particularly regarding the technical details of its operative treatment. The study aim was to describe all components of the syndrome and to evaluate the various techniques used with regards to its anatomical and functional features. METHODS: A total of 31 patients (mean age 7.4 years; range: 1.0-14.3 years) who underwent repair of VSD and AR between 1990 and 2013 was reviewed. The VSD was perimembranous in 22 patients, and subarterial in nine. Trusler's valvuloplasty technique was used in 15 patients, Yacoub's technique in seven, and Carpentier's technique (triangular resection) in four. Two patients underwent aortic valve replacement (AVR), and three patients with no significant aortic valve lesions underwent a simple patch repair of the VSD. RESULTS: The aortic valvuloplasty results were generally good, with an initial aortic valvuloplasty avoiding AVR. During the immediate postoperative period, valvuloplasty failure occurred in three patients, regardless of the technique used, and all three patients were reoperated on. The mean duration of follow up was 8.5 years (range: 3.2-20.6 years). The initial result was maintained in all patients, except for four who underwent late AVR. CONCLUSION: The study findings contributed to an analysis of VSD and AR, and helped to clarify the best surgical strategy. The results obtained suggest that adequacy of the initial repair is the most important determinant of subsequent evolution.

When coronary arteries need systolic pressure: surgical considerations.

OBJECTIVES: Patients with pulmonary atresia and intact ventricular septum (PA-IVS) may have coronary sinusoids connected to a hypertensive right ventricle. Coronary perfusion may then completely depend on the right ventricular systolic pressure wave; decompression of the right ventricle can be deleterious in these patients. This study was set to investigate the treatment strategies and outcomes of patients with coronary sinusoids. METHODS: National multicentre retrospective analysis over 15 years (1985-2010) in a population of ~10.2 million people. All patients with PA-IVS and coronary sinusoids were identified from local databases. All angiograms, echocardiograms, surgical reports and outcome data were reviewed. RESULTS: Thirty patients were identified. Right ventricular-dependent coronary circulation was present in nine patients (30%). A systemic-to-pulmonary artery shunt was created in 23 patients (77%) at a median age of 13.0 days (range: 1-479). A bidirectional Glenn was performed in 20 children at a median age of 7.8 months (range: 2.1-112.9) and 11 children proceeded to a Fontan repair at a median age of 3.6 years (range: 2.1-19.6). Pulmonary valve perforation and angioplasty were performed in six children and in three a simultaneous decompression procedure was also done. Ten patients died (33%). Four died at the time of systemic-to-pulmonary artery shunt and three during the bidirectional Glenn shunt. In all these patients myocardial ischaemia was reported. CONCLUSIONS: Mortality in PA-IVS with sinusoids is high. Right ventricular coronary dependence may lead to early death if the right ventricular systolic pressure wave is interrupted. Adequate understanding of the underlying pathology and pathophysiology is essential before attempting any procedure in this subgroup of children.

Ten-year experience with surgical treatment of adults with congenital cardiac disease.

The number of adults with congenital cardiac disease continues to increase, and adult patients are now more numerous than paediatric patients. We sought to identify risk factors for perioperative death and report our results with surgical management of adult patients with congenital cardiac disease. We retrospectively analysed in-hospital data for 244 consecutive adult patients who underwent surgical treatment of congenital cardiac disease in our centre between January, 1998 and December, 2007. The mean patient age was 27.2 plus or minus 11.9 years, 29% were in functional class III or IV, and 25% were cyanosed. Of the patients, half were operated on for the first time. A total of 61% of patients underwent curative operations, 36% a reoperation after curative treatment, and 3% a palliative operation. Overall mortality was 4.9%. Predictive factors for hospital death were functional class, cyanosis, non-sinus rhythm, a history of only palliative previous operation(s), and an indication for palliative treatment. Functional class, cyanosis, type of initial congenital cardiac disease (single ventricle and double-outlet right ventricle), and only palliative previous operation were risk factors for prolonged intensive care stay (more than 48 hours). The surgical management of adult patients with congenital cardiac disease has improved during recent decades. These generally young patients, with a complex pathology, today present a low post-operative morbidity and mortality. Patients having undergone palliative surgery and reaching adulthood without curative treatment present with an increased risk of morbidity and mortality. Univentricular hearts and double-outlet right ventricles were associated with the highest morbidity.

Right-to-left shunting with normal pulmonary pressure complicating atrial septal defect surgery.

We report on a patient in whom a right-to-left shunt with normal pulmonary arterial pressures occurred after surgery of an inferior vena caval sinus venosus-type atrial septal defect. In addition to the inferior vena cava to left atrium anatomic defect, the exceptional occurrence of a post-operative right atrial inflow stenosis contributed to the right-to-left shunting.