Impact of delayed systemic heparinization on postoperative bleeding and thromboembolism during post-cardiotomy extracorporeal membrane oxygenation in neonates.

INTRODUCTION: Veno-arterial extracorporeal membrane oxygenation is well-established for pediatric patients with post-cardiotomy heart failure. However, extracorporeal membrane oxygenation support is associated with major complications, that is, hemorrhage and thromboembolism. We seek to report our experience with delayed systemic heparinization during neonatal cardiac extracorporeal membrane oxygenation and its impact on bleeding and thromboembolism. METHODS: We retrospectively identified 15 consecutive neonates who were placed on extracorporeal membrane oxygenation after congenital heart surgery during a period of 3 years (2015-2017). Our anticoagulation protocol consisted of full heparin reversal by protamine after switching from cardiopulmonary bypass to extracorporeal membrane oxygenation (target activated clotting time: 120 ± 20 seconds). Administration of systemic heparinization was delayed until postoperative drainage volume declined to <1 mL/kg/h. Primary study endpoints were thromboembolism, bleeding, and requirement of blood products on extracorporeal membrane oxygenation. RESULTS: Our cohort (mean age: 13 ± 2.6 days; mean weight: 3.1 ± 0.3 kg; 66.7% male) required post-cardiotomy extracorporeal membrane oxygenation with a mean support time of 4.5 ± 2.2 days. Systemic heparinization was delayed averagely for 18.1 ± 9.3 hours. No thromboembolic events were observed on extracorporeal membrane oxygenation or after weaning. Relevant surgical site bleeding occurred in two patients (13.3%) requiring re-thoracotomy on the first postoperative day. Analysis of transfusion volumes revealed 24.5 ± 21.9 mL/kg/d mean packed red blood cells, 9.6 ± 7.1 mL/kg/d mean fresh frozen plasma, and 7.5 ± 5.7 mL/kg/d mean platelets. In-hospital survival was 86.6% (n = 13). CONCLUSION: In this retrospective analysis, the results of delayed systemic heparinization in neonatal post-cardiotomy extracorporeal membrane oxygenation could lead one to conclude that this routine is safe and favorable with low risk for thromboembolic events, reduced postoperative hemorrhage, and reduced blood product utilization.

Age-related reoperation rate after the Ross procedure: a report from the German Ross Registry.

BACKGROUND AND AIM OF THE STUDY: An increasing number of young adult patients are choosing bioprostheses for aortic valve replacement (AVR). In this context, the Ross operation deserves renewed consideration as an alternative biological substitute. After both the Ross procedure and bioprosthetic AVR, reoperation rates remain a concern and may be related to age at surgery. Herein are reported details of freedom from reoperation after the Ross procedure for different age groups. METHODS: The reoperation rates of 1,925 patients (1,444 males, 481 females; mean age 41.2 ± 15.3 years) from the German Ross registry with a mean follow up of 7.4 ± 4.7 years (range: 0.00-18.51 years; total 12,866.6 patient-years) were allocated to three age groups: group I < 40 years; group II 40-60 years; and group III > 60 years. RESULTS: At 10 years (respectively 15 years) of follow up, freedom from reoperation was 86% (76%) in group I, 93% (85%) in group II, and 89% (83%) in group III. CONCLUSION: There is some evidence that, at least during the first 10 and 15 years after AVR, the Ross procedure provides a significantly lower reoperation rate in young adult and middle-aged patients aged < 60 years. This information may be of interest to the patients' or physicians' decision-making for aortic valve surgery.

Autologous Tissue Technique to Repair Unilateral Isolated Absence of a Pulmonary Artery.

Isolated unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly in which one branch pulmonary artery has no connection to the main pulmonary trunk (most often there is ductal origin). Without treatment, it may lead to ipsilateral pulmonary hypoplasia and contralateral pulmonary artery hypertension. To avoid these complications, early surgical repair of UAPA is necessary. Surgical strategies include direct anastomosis between the "isolated" branch pulmonary artery (PA) and the main pulmonary trunk or creation of an interposition graft using prosthetic material or flap techniques. We describe a surgical technique using a totally autologous interposition tube graft.

A multicentre evaluation of the autograft procedure for young patients undergoing aortic valve replacement: update on the German Ross Registry†.

OBJECTIVES: Conventional aortic valve replacement (AVR) in young, active patients represents a suboptimal solution in terms of long-term survival, durability and quality of life. The aim of the present work is to present an update on the multicentre experience with the pulmonary autograft procedure in young, adult patients. METHODS: Between 1990-2013, 1779 adult patients (1339 males; 44.7 ± 11.6 years) underwent the pulmonary autograft procedure in 8 centres. All patients underwent prospective clinical and echocardiographic examinations annually. The mean follow-up was 8.3 ± 5.1 years (range 0-24.3 years) with a total cumulative follow-up of 14 288 years and 662 patients having a follow-up of at least 10 years. RESULTS: The early (30-day) mortality rate was 1.1% (n = 19). Late (>30 day) survival of the adult population was comparable with the age- and gender-matched general population (observed deaths: 101, expected deaths: 91; P = 0.29). Freedom from autograft reoperation at 5, 10 and 15 years was 96.8, 94.7 and 86.7%, respectively, whereas freedom from homograft reoperation was 97.6, 95.5 and 92.3%, respectively. The overall freedom from reoperation was 94.9, 91.1 and 82.7%, respectively. Longitudinal modelling of functional valve performance revealed a low (<5%) probability of a patient being in higher autograft regurgitation grades throughout the first decade. Similarly, excellent homograft function was observed throughout the first 15 years. CONCLUSION: The autograft principle results in postoperative long-term survival comparable with that of the age- and gender-matched general population and reoperation rates within the 1%/patient-year boundaries and should be considered in young, active patients who want to avoid the shortcomings of conventional prostheses.

Towards a Tissue-Engineered Contractile Fontan-Conduit: The Fate of Cardiac Myocytes in the Subpulmonary Circulation.

The long-term outcome of patients with single ventricles improved over time, but remains poor compared to other congenital heart lesions with biventricular circulation. Main cause for this unfavourable outcome is the unphysiological hemodynamic of the Fontan circulation, such as subnormal systemic cardiac output and increased systemic-venous pressure. To overcome this limitation, we are developing the concept of a contractile extracardiac Fontan-tunnel. In this study, we evaluated the survival and structural development of a tissue-engineered conduit under in vivo conditions. Engineered heart tissue was generated from ventricular heart cells of neonatal Wistar rats, fibrinogen and thrombin. Engineered heart tissues started beating around day 8 in vitro and remained contractile in vivo throughout the experiment. After culture for 14 days constructs were implanted around the right superior vena cava of Wistar rats (n = 12). Animals were euthanized after 7, 14, 28 and 56 days postoperatively. Hematoxylin and eosin staining showed cardiomyocytes arranged in thick bundles within the engineered heart tissue-conduit. Immunostaining of sarcomeric actin, alpha-actin and connexin 43 revealed a well -developed cardiac myocyte structure. Magnetic resonance imaging (d14, n = 3) revealed no constriction or stenosis of the superior vena cava by the constructs. Engineered heart tissues survive and contract for extended periods after implantation around the superior vena cava of rats. Generation of larger constructs is warranted to evaluate functional benefits of a contractile Fontan-conduit.

Ascending aortic aneurysm after pediatric heart transplantation: case report of an unusual complication.

A 28-month-old boy, weighing 11 kg, with severe dilated cardiomyopathy, was transplanted on December 1995. Hypertension and supraventricular tachycardia were detected in the immediate post-operative period, with favorable outcome. After 5 months of clinically asymptomatic follow-up, a dilation in the ascending aorta was observed on routine echocardiogram. Nuclear magnetic resonance imaging (NMRI) confirmed an ascending aortic aneurysm, with a diameter of 38 mm. An operation was performed, a bovine pericardium patch was sutured with reconstruction of the aortic wall, excluding the aneurysm. Good recovery was obtained and the child was discharged on Day 7 postoperatively. A post-operative echocardiogram showed absence of the aortic aneurysm and good surgical results. Another NMRI was done 5 months later, showing an intact ascending aorta. After 64 months, the patients clinical condition was confirmed as normal by echocardiogram. Surgical treatment was successful and the positive results have been maintained.

Ross Procedure in Neonates and Infants: A European Multicenter Experience.

BACKGROUND: Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability. METHODS: A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013. RESULTS: Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts. CONCLUSIONS: Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding.

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.

Heart neoplasms in children: retrospective analysis.

BACKGROUND: The advancement of noninvasive imaging methods has resulted in the increase in diagnosis of heart neoplasms. However, the literature has few studies involving primary cardiac neoplasms in children. OBJECTIVE: To retrospectively review cases of primary heart neoplasms in children, considering the initial clinical manifestations, diagnostic tests used, surgical indication, histopathological types and immediate postoperative course. METHODS: The retrospective study was based on the assessment of medical records from 1983 to 2011. Only cases that were referred for surgical treatment during that period were included. Age at admission, prenatal diagnosis, family history, initial symptoms and the results of performed tests were assessed. Moreover, the date and indication of the surgery, intraoperative findings, the result of the histopathological analysis, as well as the immediate postoperative complications were recorded. RESULTS: Of the 18 patients studied, the most frequent clinical manifestations were dyspnea and heart murmur (7 and 6 patients, respectively); the most often used adjunct diagnostic method was echocardiography (18 patients); cavitary obstruction or ventricular inflow or outflow tract obstruction was the main indication for surgery (12 cases); the most common histological profile was rhabdomyoma (7 patients) and most patients showed good clinical outcome. CONCLUSIONS: In this study, imaging diagnosis was basically attained through echocardiography, with good correlation with intraoperative findings. Histopathological findings were consistent with the literature, with rhabdomyoma being the most common neoplasm in children. The evolution after surgical treatment was favorable in most cases.

Evaluation of surgical treatment of congenital heart disease in patients aged above 16 years.

BACKGROUND: The increasing number of children with evolving congenital heart diseases demands greater preparation of professionals and institutions that handle them. OBJECTIVE: To describe the profile of patients aged over 16 years with congenital heart disease, who have undergone surgery, and analyze the risk factors that predict hospital mortality. METHOD: One thousand five hundred twenty patients (mean age 27 ± 13 years) were operated between January 1986 and December 2010. We performed a descriptive analysis of the epidemiological profile of the study population and analyzed risk factors for hospital mortality, considering the complexity score, the year in which surgery was performed, the procedure performed or not performed by the pediatric surgeon and reoperation. RESULTS: There was a significant increase in the number of cases from the year 2000. The average complexity score was 5.4 and the septal defects represented 45% of cases. Overall mortality was 7.7% and most procedures (973 or 61.9%) with greater complexity were performed by pediatric surgeons. Complexity (OR 1.5), reoperation (OR 2.17) and pediatric surgeon (OR 0.28) were independent risk factors influencing mortality. Multivariate analysis showed that the year in which the surgery was performed (OR 1.03), the complexity (OR 1.44) and the pediatric surgeon (OR 0.28) influenced the result. CONCLUSION: There is an increasing number of patients aged 16 years which, despite the large number of simple cases, the most complex ones were referred to pediatric surgeons, who had lower mortality, especially in recent years.

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.

The impact of preexisting myocardial remodeling on ventricular function early after tetralogy of Fallot repair.

BACKGROUND: Twenty-three patients (median age 23 months) who underwent Fallot's tetralogy repair were investigated prospectively to detect a possible association between histopathologic myocardial remodeling and echocardiographic findings of systolic or diastolic ventricular dysfunction. METHODS: Intraoperatively resected infundibular bands and subendocardial biopsy samples from the right ventricle (RV) and left ventricle were obtained for histopathologic evaluation. Tissue Doppler echocardiographic interrogation of the ventricles was performed before surgery and in the postoperative period. RESULTS: Histopathologic data revealed hypertrophy of the RV cardiomyocytes and increased interstitial collagen in both ventricles. Mean values of RV isovolumic acceleration decreased significantly at the third evaluation compared with the preoperative values (P = .006). RV myocardial fibrosis greater than 8.3% was associated with a probability of altered E' of at least 0.7 (odds ratio = 2.31). CONCLUSION: Preoperative histologic myocardial remodeling influenced the postoperative RV function in this group of patients with late repair. Further studies are necessary to evaluate the myocardium in younger patients and to define its influence in the long-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.

The myocardium in tetralogy of Fallot: a histological and morphometric study.

BACKGROUND: Patients with Tetralogy of Fallot frequently develop ventricular dysfunction in the postoperative period. The histological basis of this functional alteration has been scarcely studied. OBJECTIVE: To evaluate myocardial remodeling in anatomical specimens, comparing the subepicardial and subendocardial regions, especially because the subendocardial region is easily approached by means of endomyocardial biopsy. METHODS: Transmural sections of myocardium from the right ventricular (RV) inflow tract, anterior wall and infundibulum, and from the left ventricular (LV) free wall were evaluated regarding the degree of cardiomyocyte hypertrophy, vascularization and interstitial fibrosis were analyzed. RESULTS: The mean diameter of subendocardial cardiomyocytes is similar to that of subepicardial cardiomyocytes in all regions, except for the RV infundibulum, in which subendocardial cardiomyocytes are significantly larger in relation to those of the subepicardium (p=0.007). The amount of interstitial collagen is in the upper limits of normal and was similar in the subendocardial layers in comparison with the subpericardial layer of each region; however, it was greater in the inflow tract and RV anterior wall than in the LV lateral wall. The numerical density of subendocardial capillaries was similar to that of the subepicardium and was lower than the mean minus two standard deviations of normal in all regions and layers, except for the infundibulum, in which the subepicardium showed normal values and the subendocardium showed values lower than the mean minus two standard deviations. CONCLUSION: The postnatal myocardial changes in Tetralogy of Fallot are homogeneously distributed in the subepicardial and subendocardial halves of the ventricular walls, except for the infundibulum, which has peculiar remodeling characteristics and, therefore, is not representative of the other ventricular regions and layers for morphometric studies.

[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.

Pediatric heart transplantation in refractory cardiogenic shock: a critical analysis of feasibility, applicability and results.

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.

Tumores cardíacos em criança: análise retrospectiva / Heart neoplasms in children: retrospective analysis

FUNDAMENTO: Avanço dos métodos não invasivos de imagem proporcionou o aumento no número de diagnóstico de tumores cardíacos. Apesar disso, a literatura apresenta poucos trabalhos envolvendo tumores cardíacos primários em crianças. OBJETIVO: Avaliar retrospectivamente casos de tumores cardíacos primários em crianças, considerando manifestações clínicas iniciais, exames utilizados para o diagnóstico, indicação cirúrgica, tipos histopatológicos encontrados e evolução pós-operatória imediata. MÉTODOS: O estudo foi retrospectivo, baseado na avaliação de prontuários no período de 1983 a 2011. Incluímos somente casos que foram orientados para tratamento cirúrgico no período. Avaliaram-se a idade na admissão, o diagnóstico pré-natal, a história familial, os sintomas iniciais e os resultados de exames realizados. Foram coletados, ainda, a data e indicação de cirurgia, os achados intraoperatórios, o resultado do exame histopatológico, assim como as complicações imediatas no pós-operatório. RESULTADOS: Dos 18 pacientes estudados, as manifestações clínicas mais encontradas foram dispneia e sopro cardíaco (7 e 6 pacientes, respectivamente); o método de complemento diagnóstico mais usado foi o ecocardiograma (18 pacientes); a obstrução cavitária ou do trato de entrada ou saída ventricular foi a principal indicação de cirurgia (12 casos); o perfil histológico mais encontrado foi rabdomioma (7 pacientes); a maioria dos pacientes apresentou boa evolução clínica. CONCLUSÃO: Neste estudo o diagnóstico por imagem foi basicamente ecocardiográfico, com boa correlação com os achados intraoperatórios. Os achados histopatológicos foram concordantes com a literatura, com o rabdomioma apresentando-se como o tumor mais comum em crianças. A evolução após tratamento cirúrgico mostrou-se favorável na maior parte dos casos.

BACKGROUND: The advancement of noninvasive imaging methods has resulted in the increase in diagnosis of heart neoplasms. However, the literature has few studies involving primary cardiac neoplasms in children. OBJECTIVE: To retrospectively review cases of primary heart neoplasms in children, considering the initial clinical manifestations, diagnostic tests used, surgical indication, histopathological types and immediate postoperative course. METHODS: The retrospective study was based on the assessment of medical records from 1983 to 2011. Only cases that were referred for surgical treatment during that period were included. Age at admission, prenatal diagnosis, family history, initial symptoms and the results of performed tests were assessed. Moreover, the date and indication of the surgery, intraoperative findings, the result of the histopathological analysis, as well as the immediate postoperative complications were recorded. RESULTS: Of the 18 patients studied, the most frequent clinical manifestations were dyspnea and heart murmur (7 and 6 patients, respectively); the most often used adjunct diagnostic method was echocardiography (18 patients); cavitary obstruction or ventricular inflow or outflow tract obstruction was the main indication for surgery (12 cases); the most common histological profile was rhabdomyoma (7 patients) and most patients showed good clinical outcome. CONCLUSIONS: In this study, imaging diagnosis was basically attained through echocardiography, with good correlation with intraoperative findings. Histopathological findings were consistent with the literature, with rhabdomyoma being the most common neoplasm in children. The evolution after surgical treatment was favorable in most cases.