Carla Tanamati

Assessment of the pulmonary vascular blood supply in patients with pulmonary atresia, ventricular septal defect, and aortopulmonary collateral arteries

OBJECTIVE: To study the morphometric characteristics of the central pulmonary arteries and aortopulmonary collateral arteries by assessing the morphology of the pulmonary vascular blood supply, to determine their significance in surgical treatment. METHOD: From January 1990 to June 2001, 40 patients were studied. Those who had the complete cineangiocardiographic study prior to the first surgical intervention were included in the study. The morphometric characteristics of the central pulmonary arteries (PPAA) and aortopulmonary collateral arteries (APCA) were analyzed, as was the distribution of blood irrigation to the lungs. The following indices were calculated: pulmonary arterial index (PPAAI), aortopulmonary collateral arterial index (APCAI), and total neopulmonary arterial index (TNPAI = PPAAI + APCAI). The surgical treatment was considered palliative (PT), palliative definitive (PDT), and definitive (DT). RESULTS: The palliative treatment predominated. No statistically significant differences were observed in the patients undergoing PT, PDT, and DT, in regard to PPAAI, APCAI, and TNPAI. Comparing PPAAI and APCAI, no difference was observed for DT (P=0.4309); APCAI was greater than PPAAI for PT (P=0.0176); and APCAI was descriptively greater for PDT. The TNPAI of patients undergoing DT was greater than that of patients undergoing PT (P=0.0959). Five morphologically similar subgroups were identified and designated as B1, B2, B3, B4, and B5. Overall mortality was 17.5%. CONCLUSION: The morphometric characteristics are important, but the morphology of the pulmonary vascular blood supply of the PPAA and APCA proved to be better for guiding the surgical treatment. Independently of the didactical division into subgroups, PT predominated. Mortality was not correlated with the morphometric characteristics.

Transxiphoid approach without median sternotomy for the repair of atrial septal defects.

BACKGROUND Interest in minimally invasive procedures has recently increased because it results in less surgical trauma, decreased patient discomfort, short hospital stay, reduced costs, and better cosmetic appearance. Based on these facts, we have been using the transxiphoid process approach without sternotomy for the correction of atrial septal defects. METHODS From July 1996 to January 1997, the xiphoid process window approach was performed in 10 patients with ostium secundum atrial septal defect. Ages ranged from 6 months to 14 years (mean, 5.3 years). In all patients, extracorporeal circulation was carried out by means of cannulation of the femoral artery and both caval veins and of aortic cross-clamping. Videothoracoscopy was used to improve visualization of the aorta. RESULTS There were no intraoperative or postoperative complications, and in all but 1 patient, extubation was possible while in the operating room. CONCLUSIONS The xiphoid process window, with no median sternotomy, permitted closure of the atrial septal defects with good results and could be used as a less invasive technique for their correction.

Intraventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect: Advantages of multiple patches

OBJECTIVE The objective of this paper is to report our experience with biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect by means of multiple patches that simplify and render feasible the intraventricular correction of this complex anomaly. METHODS From April 1987 to April 1999, in 18 patients with double-outlet right ventricle and noncommitted ventricular septal defect, a technical modification that used multiple patches of bovine pericardium was used to construct an intraventricular tunnel connecting the left ventricle to the aorta. Ages ranged from 2 months to 13 years (mean age 4.73 +/- 3.41 years). RESULTS The early mortality was of 11.1% (2 patients). Surviving patients were followed up for a mean of 2.65 years. Three late deaths (16.6%) occurred: 5 months, 7 months, and 7 months after the operation. All but 1 patient are in New York Heart Association class I. CONCLUSION The use of multiple patches for biventricular correction of this anomaly simplifies and renders feasible the intraventricular repair in cases in which the 1-patch technique was deemed impossible.

Congenital atresia of the ostium of the left coronary artery. Diagnostic difficulty and successful surgical revascularization in two patients

We report two cases of congenital atresia of the ostium of the left coronary artery. Case 1: a six-month-old infant presenting with serious cardiac insufficiency. A noninvasive diagnosis of dilated myocardiopathy was established and the clinical picture was pharmacologically compensated. When the patient was nine months of age, a hemodynamic study was performed that revealed congenital atresia of the ostium of the left coronary artery; the infant immediately underwent a successful anastomosis of the internal mammary artery with the left coronary artery. Case 2: an eleven-year-old asymptomatic boy with a history of heart murmur from the age of six months on, was referred for surgery with a diagnosis of anomalous origin of the left coronary artery from pulmonary trunk. A definitive diagnosis of atresia of the left coronary ostium was only established during surgery. Successful surgical revascularization with the left internal mammary artery, and left ventricular aneurysmectomy were performed.

Alternative Nonvalved Techniques for Repair of Truncus Arteriosus: Long-Term Results

Primary early repair has been advocated as the preferred surgical approach to truncus arteriosus in infancy. This approach usually includes the reconstruction of the right ventricle outflow tract using a valved extracardiac conduit. However, the longevity of these conduits and the risk of their replacement have been a subject of major concern. When used in early infancy, these conduits require repeated replacement because of internal obstruction or because the patient has outgrown the conduit. Our group first described the possibility of correction without an extracardiac conduit in 1990, although it has been performed at our institution since 1987. The ideal candidates for this type of correction include patients up to 5 months of age who have truncus arteriosus type I or II (Colllet-Edwards classification), no anomalous trajectory of the coronary artery crossing anteriorly to the truncus, and no significant pulmonary vascular resistance (pulmonary vascular resistance <7 Wood units). This surgical technique is described and the outcomes reviewed. Copyright 1999 by W.B. Saunders Company

Transplante cardíaco pediátrico em vigência de choque cardiogênico refratário: análise crítica da viabilidade, aplicabilidade e resultados

BACKGROUND In children with dilated cardiomyopathy who are on the waiting list for heart transplantation, we evaluate the seriousness of their hemodynamic conditions. Some develop cardiogenic shock, and the mortality rate is high. Even with inotropic and respiratory support, heart transplantation is considered an extremely grave circumstance. OBJECTIVE The objective of this study is to report on our experience with children in this condition, in an attempt to analyze the viability, applicability and results of heart transplantation in these children. METHODS From March 2001 to February 2004, 22 children with dilated cardiomyopathy who were on the waiting list for heart transplantation developed cardiogenic shock, requiring transfer to pediatric intensive care unit (ICU), intubation and inotropic support. Their ages ranged from 11 months to 11 years (mean age: 4.3 years), 55% were males, 14 could be listed as clinical priority, and the remaining 8 were removed from the waiting list due to their unfavorable clinical conditions. RESULTS Eight heart transplantations were performed, and 6 children died while on the waiting list (42.9%). Two children died (25%) after transplantation and the remaining 6 were discharged from hospital in good clinical condition. The two main complications were organ rejection in 4 cases and infection in 5 cases. Two patients developed neurological complications, and one of them fully recovered. CONCLUSION Children with cardiomyopathy and cardiogenic shock require immediate heart transplantation; only 57.1% could be transplanted, with an early 25% mortality rate. Those who survived transplantation showed good clinical progress, similar to that of children transplanted on an elective basis.

Calcified amorphous tumor of the heart: case report

Calcified amorphous tumor of the heart consists of a cardiac mass of rare nonneoplastic nature that mimics malignancy and causes symptoms due to obstruction or embolization of calcific fragments. We present a case of tumor 17-year-old young, male, in tricuspid valve, with classic pathological findings. It was preferred to approach for classic median esternotomy, installation of the circuit of extracorporal circulation and right atriotomy, exereses of tumor, DeVegas plasty in tricuspid valve and bicuspidization. The amatomopathological study demonstrated presence of extensive calcification and metaplastic bone areas. The patient had an uneventful hospitalization.

Isolated left ventricular noncompaction: unusual cause of decompensated heart failure and indication of heart transplantation in the early infancy: case report and literature review

A female infant, aged 10 months, arrived at our hospi-tal and was admitted with clinical signs of heart failure anda history of a previous admission at Intensive Care Unitfor decompensated heart failure and cardiac arrest onemonth before. She was born at term of healthy, unrelatedparents and was asymptomatic until the age of two months,when she presented with shortness of breath, poor appe-tite and weight loss. She weighed 6.8 Kg and was 75.5 cmtall. Cardiovascular examination revealed a decreased-vol-ume peripheral pulse and a third heart sound. There wasalso hepatomegaly, 4 cm below the right costal margin. Nodysmorphic features were observed. There was no sign ofarrhythmia noted while awaiting transplantation.The patient evolved with refractory heart failure as aresult of severely deteriorated left ventricular function andbecame a candidate for heart transplantation. On admis-sion, the infant’s medications were aldactone 1.5 mg/kg/day, captopril 1.5 mg/kg/day, furosemide 3.5 mg/kg/dayand digoxin 10 mg/kg/day.During the pre-transplantation exams, the chest X-rayrevealed substantial cardiomegaly. The electrocardiographyshowed left ventricular overload, but no signs of ventricu-lar arrhythmias. The echocardiogram showed dilated car-diac chambers (important enlargement of left atrium andleft ventricle). There was a severe left ventricular systolicdysfunction (shortening fraction of 7.3%) and a discrete leftatrioventricular valve insufficiency, with no signs of throm-bosis. The dimension of the septum and left ventricular wallwas 6 mm thick. Gallium-67 scintigraphy for myocardialinflammatory process was negative. An endomyocardialbiopsy revealed no evidence of myocarditis. Cardiac cath-eterization showed a systolic pulmonary pressure of 28mmHg, diastolic pulmonary pressure of 18 mmHg, meanpulmonary pressure of 21 mmHg, capillary wedge pressureof 15 mmHg, pulmonary vascular resistance index of 1.8Woods/m

Agenesia isolada da artéria pulmonar direita ou esquerda: avaliação da evolução natural e a longo prazo, após intervenção corretiva

OBJECTIVE Unilateral pulmonary artery agenesis is an uncommon isolated anomaly and since 1978 only 119 cases have been reported. In general the condition presents as pulmonary hypertension (PH) in children and hemoptysis in adults. Interventions such as pulmonary artery reconstruction and lobectomies were performed in 17% of the cases. We analyzed four of these cases, two in natural evolution and two with late term PH regression after surgical correction. METHODS Three 22,10 and 35 month old male children and one 20 month old female child were included in the study. The 22 month old presented right-sided heart failure (RHF) and cyanosis; the 10 month old presented RHF and the other two presented exertion fatigue. All had PH symptoms, right ventricular strain on the EKG and cardiomegaly. Cardiac catheterization showed systemic pressures in the contralateral pulmonary artery, with right-sided agenesis in three of the children and left-sided agenesis in one child. RESULTS Surgical correction of pulmonary artery continuity was possible in the 22 month old and 10 month old using a 7 mm diameter Goretex conduit between the pulmonary arteries up to the hypoplastic contralateral pulmonary hilum. There was early and late regression of the PH signs and the children remained stable during follow-up to the ages of 7 and 2.5 years, respectively. The pressure ratio between the left and right ventricles was 30 and 40%, in both cases. Pulmonary perfusion increased from 8 to 44% and from 8 to 23%, in the two cases. The same procedure was scheduled for the other patients. CONCLUSION This technique has become the operation of choice for similar cases, that are rarely described in literature, even in the presence of severe PH and contralateral pulmonary artery hypoplasia.

Post-cardiotomy ECMO in pediatric and congenital heart surgery: impact of team training and equipment in the results.

Introduction Post-cardiotomy myocardial dysfunction requiring mechanical circulatory support occurs in about 0.5% of cases. In our environment, the use of extracorporeal membrane oxygenation has been increasing in recent years. Objective To evaluate the impact of investment in professional training and improvement of equipment in the rate of weaning from extracorporeal membrane oxygenation and survival. Methods A retrospective study. Fifty-six pediatric and/or congenital heart patients underwent post-cardiotomy extracorporeal membrane oxygenation at our institution between November 1999 and July 2014. We divided this period into two phases: phase I, 36 cases (before the structuring of the extracorporeal membrane oxygenation program) and phase II, 20 cases (after the extracorporeal membrane oxygenation program implementation) with investment in training and equipment). Were considered as primary outcomes: extracorporeal membrane oxygenation weaning and survival to hospital discharge. The results in both phases were compared using Chi-square test. To identify the impact of the different variables we used binary logistic regression analysis. Results Groups were comparable. In phase I, 9 patients (25%) were weaned from extracorporeal membrane oxygenation, but only 2 (5.5%) were discharged. In phase II, extracorporeal membrane oxygenation was used in 20 patients, weaning was possible in 17 (85%), with 9 (45%) hospital discharges (P<0.01). When the impact of several variables on discharge and weaning of extracorporeal membrane oxygenation was analyzed, we observe that phase II was an independent predictor of better results (P<0.001) and need for left cavities drainage was associated with worse survival (P=0.045). Conclusion The investment in professional training and improvement of equipment significantly increased extracorporeal membrane oxygenation results.