Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve. The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.

Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.

First use of hypertonic saline dextran in children: a study in safety and effectiveness for atrial septal defect surgery.

Hypertonic saline dextran (7.5% NaCl + 6% Dextran-70) has been used in adults in several studies and shown beneficial effects in hypovolemic shock, trauma, cardiogenic shock, and cardiac surgery. There have never been studies of this solution in children. This work studies its effect in children undergoing surgery for the correction of atrial septal defects. Twenty-five children underwent correction of atrial septal defect using cardiopulmonary bypass with bloodless priming. Children were divided in five groups and each received an incremental hypertonic saline dextran dose of 0.1, 0.5, 1.0, 2.0, and 4.0 mL/kg, 5 min before the beginning of cardiopulmonary bypass. Collected data were fluid balance, amount of bleeding, blood/derivative transfusion occurrence, plasma sodium, and hematocrit. Patients were divided into low-dose (0-1 mL/kg) and high-dose (2-4 mL/kg) groups. Analysis of variance was used to determine differences in blood loss between groups. The fluid balance and blood/derivative requirements were compared through Student's t test and Fisher's exact test (2-tail), respectively. All patients were discharged from hospital with corrected atrial septal defect. No hypertonic saline dextran-related complications occurred. There were no differences in the amount of bleeding. The high-dose group exhibited a significant decrease in fluid balance and in blood/derivative requirements in comparison with the low-dose group. In this study, the use of hypertonic saline dextran in the pediatric population submitted to cardiopulmonary bypass is safe and does not raise the amount of bleeding. Its effective doses produce negative fluid balance and reduce blood/derivative requirements.

Evolutional aspects of children and adolescents with surgically corrected aortic coarctation: clinical, echocardiographic, and magnetic resonance image analysis of 113 patients.

OBJECTIVE: The goals of this study were to determine the prevalence of hypertension and recoarctation in operated children and teenagers and to integrate clinical and imaging technique data. METHODS: One hundred thirteen infants and children (ages 14 years or less) were retrospectively divided into 3 groups according to the age at operation and the surgical technique: 79 underwent resection with end-to-end anastomosis; 14 had patch enlargement; 13 had subclavian flap aortoplasty; and 7 had other techniques performed. The mean age at operation was 3.95 +/- 4.17 years and the mean follow-up period was 4.62 +/- 4.90 years. Each patient was clinically examined and Doppler echocardiography was performed in 112 patients. Sixty-six patients underwent magnetic resonance imaging. Diameters of aortic arch were measured at 4 levels; the ratios between each one and descendent aorta diameters were calculated. Qualitative variables and associations were studied by Fisher exact test or chi-squared test. Comparisons of measurements in different groups were performed using variance analysis, with tests of selective contrasts (nonparametric tests). The level of statistical significance was <.05. RESULTS: The prevalence of hypertension and recoarctation was 38% and 14%, respectively. No statistical difference was found among the age groups. In 65 patients who underwent magnetic resonance imaging, the transverse aortic arch was hypoplastic in 31 (47%) patients; 41 (63%) had stenosis at the site of anastomosis (ratio < 0.9). CONCLUSION: Hypoplasia of transverse aortic arch was highly prevalent. These data suggest that hypoplastic aortic arch should be corrected concomitantly with coarctation.

Long-term results of correction of tetralogy of Fallot in adulthood.

OBJECTIVES: The natural history of tetralogy of Fallot (TOF) allows that a minority of patients reach adulthood without any treatment, representing mild forms of the disease. The aim of this study is the long-term evaluation of patients with TOF surgically treated in adulthood, in order to define its real benefit. METHODS: Between November 1982 and January 2001, 39 patients older than 18 years of age with tetralogy of Fallot underwent total correction. Mean age was 26.6 years (range 18-67) and 21 patients (53.8%) were females. A previous modified Blalock-Taussig shunt was performed in four patients (10.3%). Fifteen patients (38.5%) were in NYHA functional class III or IV. Mean hematocrit was 53.6+/-10% and the mean gradient across the right-ventricular outflow tract was 93.9+/-24.8 mmHg. The operation was performed via transatrial/transpulmonary approach in 16 patients (41%) and six patients (15.4%) required transannular patch. Pulmonary valvotomy was necessary in 13 patients (33.3%) and pulmonary valve replacement with bioprosthesis in 3 patients (7.7%). RESULTS: Hospital and late mortality were 5.1 and 7.7%, respectively. The mean follow-up was 45.1 months (range 1-194 months). Actuarial survival was 91.2+/-4.9%, 85.5+/-7.2% and 68.4+/-16.3% at 3, 7 and 15 years, respectively. In the latest follow-up, 27 (79.4%) of the survivals are presently in NYHA functional class I (P<0.001). Echocardiography has shown moderate/severe pulmonary insufficiency in 9 patients (26.5%), moderate pulmonary stenosis in 3 patients (8.8%) and residual ventricular septal defect in 4 patients (11.8%). Arrhythmias were identified in 38.9% of patients with symptoms suspicious of rhythm disturbances. There was impairment of right-ventricular function in 13 patients (38.2%). Three patients were reoperated on to close residual ventricular septal defects in two patients and for pulmonary valve replacement in one patient. CONCLUSIONS: The overall survival of surgically treated adult patients with TOF is acceptable. The great benefit of the complete repair at this age is the functional improvement. On the other hand, late complications closely related to chronic hypoxia, such as arrhythmia and ventricular dysfunction might direct for a more careful follow-up after the surgical correction.

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

[Total anomalous drainage of the pulmonary veins-- surgical therapy for the infradiaphragmatic and mixed anatomical types]. / Drenagem anômala total das veias pulmonares: terapêutica cirúrgica dos tipos anatômicos infracardíaco e misto.

OBJECTIVE: To study the in-hospital evolution of patients with the infradiaphragmatic and mixed types of total anomalous pulmonary venous drainage (TAPVD), who underwent surgical therapy. METHODS: Of the 65 patients diagnosed with isolated TAPVD and operated on from December 1993 to March 2002, 7 (10.8%) patients with the mixed and infradiaphragmatic forms were retrospectively selected. Their ages ranged from 5 days to 19 months (mean of 7 months), 5 (71.4%) were males, and their clinical diagnosis was established with 2-dimensional echocardiography. Four (57.1%) patients had the mixed form, which was intrinsic obstructive in 1 patient, with mild stenosis of the left inferior vein. The remaining 3 (42.9%) patients had the obstructive infradiaphragmatic form, extrinsic at the level of the diaphragm. All surgeries were performed through median sternotomy with hypothermic extracorporeal circulation, and total circulatory arrest was required in 2 patients. RESULTS: In-hospital death occurred in 1 patient with infradiaphragmatic TAPVD with connection of the inferior vertical vein with the portal vein. The cause of death was related to multisystem organ failure. In 4 (57.1%) patients, the postoperative period was characterized by the presence of low cardiac output and pulmonary hypertension. CONCLUSION: The result of the surgical correction of this anomaly is associated with acceptable morbidity and mortality, depending on early referral and surgery, without progression of the pulmonary vascular hypertension findings.

Fontan operation and the cavopulmonary technique: immediate and late results according to the presence of atrial fenestration.

OBJECTIVE: To compare immediate and late results in patients with or without fenestration who underwent cavopulmonary anastomosis so that we could assess the efficiency of the technique. METHODS: Sixty-two patients underwent surgery between 1988 and 1999, 41 with fenestration (group I -G I) and 21 without fenestration (group II -G II). Tricuspid atresia was prevalent in group I (23-56%) and single ventricle was prevalent in group II (14-66%). Mean ages at the time of operation were 7.3 years in group I and 7.6 in group II. At late follow-up, mean ages were 10.6 years in group I and 12.8 years in group II. RESULTS: Immediate and late mortality were 7.3% in G-I and 4.7% in G-II. Significant pleural effusion occurred in 41.4% of G-I patients and in 23.8% of G-II patients. Significant pericardial effusion occurred in 29.2% and 14.2%, respectively, in groups I and II. Central venous pressure was greater in G-II, 17.7 cm in H2O, as opposed to 15 cm in G-I. Hospital stay was similar between the groups, 26.3 and 21.8 days, respectively. Cyanosis and arterial insaturation occurred in 5 patients, and 4 patients were in functional class II, all from G-I. At late follow-up, 58 (93.5%) were in functional class I. Sinus rhythm was present in 94%, and pulmonary perfusion was similar in both groups. Eleven patients who underwent spirometry had good tolerance to physical effort. CONCLUSION: Atrial fenestration did not improve the immediate or late follow-up of patients who underwent cavopulmonary anastomosis, and is, therefore, dispensable.

Surgical treatment of patent ductus arteriosus in adults.

OBJECTIVE: To analyze 34 patients submitted to surgical treatment of patent arterial duct with age beyond 18 years old. METHODS: Retrospective data collected from patient's charts with more than eighteen years old, submitted to surgical correction of patent arterial duct between 1997 and 2008 at Instituto do Coração da Faculdade de Medicina da Universidade de São Paulo. RESULTS: The mean age was 28.7 (18 a 53) years and 22 (64.7%) were female. The more prevalent symptom was dyspnea (76.5%). Left lateral thoracotomy was used in 33 (97.1%); the DA was sectioned and sutured in 25 (73.5%) cases and one patient needed cardiopulmonary bypass support. There were eight (23.5%) calcified arterial duct and 12 (35.3%) previous treatment with transcatheter devices were performed. The complication rate was 32%, with one (2.9%) permanent vocal cord palsy. Two (5.8%) patients had residual shunt less than 2mm. Transient left cord voice palsy was observed in 3 (8.8%) The procedure improves functional class (P< 0.0001) and no mortality was observed. CONCLUSION: In this series, the surgical treatment of patent arterial duct in adults could be done without mortality and low incidence of complications.

[B-type natriuretic peptide assessment in the diagnosis of rejection after pediatric heart transplant].

BACKGROUND: Rejection is one of the major causes of mortality following pediatric heart transplant. B-type natriuretic peptide (BNP) has been studied as a method for the diagnosis of acute rejection, especially in adult patients undergoing heart transplant. OBJECTIVE: To correlate serum BNP levels with acute rejection as diagnosed by endomyocardial biopsy in patients of the pediatric heart transplant group. METHODS: A total of 50 BNP samples were collected from 33 children in the postoperative period of heart transplant, and data on age, gender, skin color, blood group, immune panel, follow-up time after transplant, functional class, immunosuppressive regimen used and number of rejections were analyzed. RESULTS: Thirty three children with median age of 10.13 years were analyzed; of these, 54% were females and 78% were Caucasians. BNP levels were determined at a mean time from transplant of 4.25 years. Nine episodes of rejection were diagnosed in eight patients (27%) by means of endomyocardial biopsy; of these, three were grade 3A, five were grade 2, and one had humoral rejection. At the moment of biopsy, most patients were asymptomatic. The mean serum BNP level was 77.18 pg/ml, with 144.22 pg/ml in the group with rejection and 62.46 pg/ml in the group without rejection, with p = 0.02. CONCLUSION: Asymptomatic children can present acute rejection in the postoperative period of heart transplant. Serum BNP levels show a statistically significant difference in the group with rejection and thus can be an additional method in the diagnosis of cardiac rejection.

Outcomes of aortic coarctation surgical treatment in adults.

OBJECTIVE: The aim of this study is to describe our experience in aortic coarctation surgery in adult patients by assessing the immediate and mid-term outcomes. METHODS: From January 1987 to March 2000, 50 consecutive adult patients underwent surgery for correction of aortic coarctation, through left lateral thoracotomy. Of these, forty two (84%) patients presented high blood pressure, with mean systolic arterial pressure of 170.56 mmHg (125-220 mmHg). The mean of pressure gradient in the coarctation area was 51.4 mmHg (18-123 mmHg). Other associated surgical cardiovascular diseases were not treated in the same operative act, except in two cases of patent ductus arteriosus (PDA). Three different techniques were used: aortic coarctation resection with end-to-end anastomosis was performed in 20 (40%) patients, coarctation enlargement with bovine pericardial patch was performed in 22 (44%) patients and synthetic tube interposition was performed in eight (16%) patients. RESULTS: Operative morbidity was low; there was one case of bleeding who required reoperation. The most common immediate postoperative event was high blood pressure (98%), but it was easily controlled by intravenous drugs. There was no hospital death. Mean residual pressure gradient was 18.7 (8-33 mmHg). Patients were discharged in 9.5 days (5-30). Postoperative follow-up mean was 46.8 months (1-145 months) in 45 (91.8%) patients. Forty one (91.1%) of these followed-up patients had normal blood pressure, whereas 75.6% of them without drugs intake. 93.3% of these followed-up patients were asymptomatic. Four of them required further surgical operation, one needed a pacemaker implant, other two patients needed a cardiac valve replacement and one had endocarditis. There was one related death due to sepsis secondary to endocarditis. CONCLUSION: Surgical treatment of aortic coarctation, even in adult patients, is an efficient therapeutic choice, regardless of the applied surgical technique, with low morbidity and mortality. It reduces efficiently the arterial pressure levels in both immediate and mid-term follow-up.

Off-pump correction of coronary fistula: the single suture technique approach.

The single suture technique was developed to obtain stabilization and exposure of all all coronary branches during off-pump coronary artery bypass, while maintaining hemodynamic stabilily during the procedure. We describe the use of this technique during an off-pump correction of a coronary artery fistula associated with right coronary dilatation. The suggested technique is quicker and less complex than on-pump surgery. Furthermore, it can be a useful tool for congenital fistula correction in select cases, stimulating the practice of less invasive heart surgery in these patients.

[Intrapericardic surgical treatment of teratoma in an infant]. / Tratamento cirúrgico de teratoma intrapericárdico em lactente.

The intrapericardic cardiac tumors are infrequent; however, the clinical manifestations can be serious, even with symptoms of low cardiac output or cardiogenic shock, depending on the localization of the tumor. We report the case of a 3-month-old infant who progressed to cardiogenic shock due to an intrapericardic tumor compressing the right atrium and the vena cava superior. Emergent surgery for resection of the tumor mass was recommended. The patient had a 6-month uneventful postoperative course.

A novel adjustable pulmonary artery banding system for hypoplastic left heart syndrome.

OBJECTIVE: Hypoplastic left heart syndrome remains a challenge for worldwide surgeons. Initial palliation employing bilateral pulmonary artery banding along with ductal stent implantation and atrial septostomy has been proposed as an alternative approach. However, the surgically placed bands are fixed and may become inadequate after sternum closure or with somatic growth of the patient. We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini banding system that allows for fine percutaneous adjustments of pulmonary blood flow. METHOD: Through a mid sternotomy, a 5 day-old neonate underwent bilateral pulmonary artery banding using this new system combined with placement of a main pulmonary artery to innominate artery shunt. RESULTS: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75%-85% range. On the 48th day of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. The Norwood operation and the bidirectional Glenn shunt were carried out on the 106th day of life. The bands were removed with no distortion of the pulmonary arteries. CONCLUSIONS: The clinical use of this innovative pulmonary artery banding system was feasible, safe and effective. It allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results.

OBJECTIVES: We sought to describe a new technique for tricuspid valve repair in Ebstein's anomaly and to report early echocardiographic results, as well as early and midterm clinical outcomes. METHODS: From November 1993 through August 2005, 40 consecutive patients with Ebstein's anomaly (mean age, 16.8 +/- 12.3 years) underwent a new surgical repair modified from Carpentier's procedure, the principal details of which are as follows. The anterior and posterior tricuspid valve leaflets are mobilized from their anomalous attachments in the right ventricle, and the free edge of this complex is rotated clockwise to be sutured to the septal border of the anterior leaflet, thus creating a cone the vertex of which remains fixed at the right ventricular apex and the base of which is sutured to the true tricuspid valve annulus level. Additionally, the septal leaflet is incorporated into the cone wall whenever possible, and the atrial septal defect is closed in a valved fashion. RESULTS: There was 1 (2.5%) hospital death and 1 late death. Early postoperative echocardiograms have shown good right ventricular morphology and reduction in tricuspid regurgitation grade from 3.6 +/- 0.5 to 1.2 +/- 0.5 (P < .0001). After mean follow-up of 4 years, the functional class (New York Heart Association) improved from 2.6 +/- 0.7 to 1.2 +/- 0.4 (P < .0001). Two patients required late tricuspid valve re-repair, and there was neither atrioventricular block nor tricuspid valve replacement at any time. CONCLUSIONS: This surgical technique for Ebstein's anomaly can be performed with low mortality and morbidity. Early echocardiograms showed significant reduction of tricuspid insufficiency, and the follow-up showed improvement in patients' clinical status and low incidence of reoperation.

Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery.

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.