Retrospective analysis of percutaneous intervention of the renal artery in transplanted kidneys in children and adolescents at a tertiary public hospital.

BACKGROUND: This study evaluated the long-term effects of percutaneous intervention in children and adolescents with transplant renal artery stenosis (TRAS). METHODS: Twenty patients had significant stenosis (>50%) and underwent percutaneous transluminal angioplasty (PTA/stenting) (TRAS group-intervention); 14 TNS (non-significant group -control) patients did not have significant stenosis (≤50%) and were treated clinically. The combined primary endpoints were death from all causes and late graft failure. The secondary endpoints were serum creatinine (SCr), systolic blood pressure (SBP), and diastolic blood pressure (DBP). RESULTS: No statistically significant difference was found between TRAS-Intervention(N = 20) and TNS groups-Control (N = 14) for these clinical parameters: deaths, 1 (5.0%) vs. 0 (0.0%) (p = 1.000) and graft loss, 4 (20.0%) vs. 2 (14.3%) (p = 1.000). For the secondary endpoints, after 1 month and 1 year the values of SCr, SBP, and DBP were similar between the two groups but not statistically significant. DISCUSSION: In the TRAS group (intervention), the stent implantation was beneficial for treating refractory hypertension and reducing blood pressure (BP) in children and adolescents. Despite the outcomes being similar in the two groups, it can be inferred that the patients in the TRAS group (intervention) would have had a worse outcome without the percutaneous intervention. CONCLUSION: TRAS treatment with stenting can be considered for children and adolescents. Because the sample in the present study comprised of only a specific population, further studies are needed for generalization. TRIAL REGISTRATION: The trial was registered at clinictrials.gov with trial registration number NCT04225338.

Tumores cardíacos pediátricos: características de imagens ecocardiográficas / Pediatric cardiac tumors: echocardiographic imaging features

Tumores cardíacos (TC) em crianças são lesões muito raras, mas seu diagnóstico é fundamental para a conduta adotada para o paciente. A ecocardiografia é a modalidade de imagem cardiovascular mais utilizada na prática clínica para o diagnóstico inicial de TC em pacientes pediátricos. Conhecer as características ecocardiográficas das TCs pode possibilitar um diagnóstico cada vez mais precoce e a identificação de sua etiologia mais provável. Os TCs primários e benignos são os mais frequentes na população pediátrica. Entre os TCs benignos, os mais frequentes em fetos e neonatos são rabdomiomas e teratomas. Em crianças e adolescentes, rabdomiomas e fibromas são os mais comuns. Neste artigo, descrevemos as características ecocardiográficas dos principais TCs em idades pediátricas.(AU)

Cardiac tumors (CTs) in children are very rare, but their diagnosis is crucial for patient management. Echocardiography is the most commonly used cardiovascular imaging modality in clinical practice for the initial diagnosis of CTs in pediatric patients. Knowing the echocardiographic characteristics of CTs can enable an increasingly early diagnosis and the identification of its most likely etiology. Primary and benign CTs are the most frequent types in the pediatric population. Among benign CTs, the most frequent in fetuses and neonates are rhabdomyomas and teratomas. In children and adolescents, rhabdomyomas and fibromas are more common. Here we describe the echocardiographic characteristics of the most common CTs in pediatric patients.(AU)

Reference values for left and right ventricular systolic-to-diastolic duration ratio (SDR) found using both spectral and tissue Doppler of fetal heart between 20 and 36+6 weeks of gestation.

PURPOSE: To establish reference values for the systolic-to-diastolic duration ratio (SDR) of the left ventricle (LV) using spectral Doppler, as well as for the SDR' of the interventricular septum (SEP), LV, and right ventricles (RV) using tissue Doppler of the fetal heart. METHOD: This prospective and cross-sectional study evaluated 374 low-risk singleton pregnancies from 20 to 36 + 6 weeks of gestation. The ventricular filling time (FT) was obtained from LV inflow using spectral Doppler. Tissue Doppler was used to assess the FT of each ventricle by placing the cursor at the atrioventricular junction marked by the mitral and tricuspid valves, respectively. SDR was calculated as the sum of the isovolumic contraction time (ICT) and the ejection time (ET) divided by the sum of the isovolumic relaxation time (IRT) and the ventricular FT. We used regression analysis to obtain the best-fit model polynomial equation for the parameters. The concordance correlation coefficient (CCC) was used to assess intra- and inter-observer reproducibility. RESULTS: SDR and SDR' LV showed a progressive decrease with gestational age (GA); the SDR' RV and SDR' SEP did not show a significant decrease with advancing GA. The SDR LV (r = 0.29, p < 0.0001), SDR' RV (r = 0.21, p < 0.0001), SDR' LV (r = 0.20, p = 0.0001), and SDR' SEP (r = 0.25, p < 0.0001) showed a significant weak positive correlation with fetal heart rate. The inter-observer SDR' SEP measurements demonstrated poor reproducibility (CCC: 0.50), whereas intra-observer SRD LV measurements demonstrated moderate reproducibility (CCC: 0.78). CONCLUSIONS: Reference values for SDR SEP, LV, and RV using spectral and tissue Doppler of fetal heart were established between 20 and 36+6 weeks of gestation.

Use of selexipag in a teenage patient with pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a chronic, progressive, multifactorial disease. Currently, combination therapy is an attractive option for PAH management because three pathways (endothelin, nitric oxide, and prostacyclin) are involved in this disease. Selexipag is a novel oral prostacyclin pathway drug and is a highly selective IP prostacyclin receptor agonist with vasodilatory and antiproliferative effects. We report the case of a teenage patient with idiopathic PAH who presented in World Health Organization functional Class IV and showed no clinical improvement with dual therapy. We added oral selexipag to the treatment regimen and observed substantial improvement in her quality of life at the short-time follow-up. Despite the lack of childhood data regarding the use of selexipag in pediatric patients with PAH, the use of this drug in the current teenage patient improved her quality of life and exercise capacity.

Fetal cardiac function by mitral and tricuspid annular plane systolic excursion using spatio-temporal image correlation M-mode and left cardiac output in fetuses of pregestational diabetic mothers.

OBJECTIVE: To assess the mitral and tricuspid annular plane systolic excursions (MAPSE and TAPSE, respectively) and cardiac output (CO) in fetuses of pregnant women with pregestational diabetes mellitus (DM) using spatio-temporal image correlation M-mode (STIC-M) and virtual organ computer-aided analysis (VOCAL). METHODS: This study was prospective and cross-sectional. It included 45 fetuses each from mothers with pregestational DM and healthy mothers, with gestation ages ranging from 20 to 36.6 weeks. The fetal cardiac volumes were obtained and analyzed by STIC and VOCAL methods. MAPSE and TAPSE were measured by STIC-M in the apical or basal fourchamber view. The values of the right (RV) and left ventricular (LV) CO were calculated by STIC and VOCAL. RESULTS: The median values of TAPSE were 6.1 and 6.2 mm in the diabetic and control groups (P<0.001), respectively. The median values of MAPSE were 4.6 mm in the fetuses of mothers with diabetes and 4.8 mm in fetuses of healthy mothers. The fetal LV CO (60.4 L/min vs. 71.1 L/min; P=0.033, respectively) and RV CO (65.2 vs. 70.1 L/min; P=0.026, respectively) were lower in the pregestational DM group than in the control group. A significant effect of pregestational DM was observed in all functional parameters after adjusting, with fetal heart rate as covariant. There was moderate significant positive correlation between MAPSE and LV CO (r=0.53; P=0.0001) and between TAPSE and RV CO (r=0.46; P=0.0001). CONCLUSION: Significant difference in functional parameters (TAPSE, MAPSE and LV CO) obtained by STIC and VOCAL were observed in the fetuses of the pregestational DM group compared to those of the control group.

Reference ranges for the left ventricle modified myocardial performance index, respective time periods, and atrioventricular peak velocities between 20 and 36 + 6 weeks of gestation.

Objective: To establish reference ranges for the fetal left ventricle (LV) modified myocardial performance index (Mod-MPI), respective time periods, and right and left atrioventricular peak velocities between 20 and 36 + 6 weeks of gestation.Methods: This cross-sectional study evaluated 360 low-risk singleton pregnancies between 20 and 36 + 6 weeks of gestation. The LV Mod-MPI was calculated as (isovolumetric contraction time + isovolumetric relaxation time)/ejection time. Polynomial regression was used to obtain the best-fit using Mod-MPI and atrioventricular peak velocity measurements and gestational age (GA) with adjustments using the coefficient of determination (R2). The intra- and interobserver reliability was evaluated using the concordance correlation coefficient (CCC).Results: LV Mod-MPI (R2 = 0.026, p = .002) and isovolumetric relaxation time (IRT) (R2 = 0.036, p < .001) significantly increased with advancing GA. Isovolumetric contraction time (ICT) and ejection time (ET) did not significantly change with GA. RV and LV E wave, A wave, and E/A ratio significantly increased with GA (p < .001). The mean of each parameter ranged as follows: LV Mod-MPI (0.44-0.47 s), IRT (0.041-0.045 s), ICT (0.032-0.034 s), ET (0.167-0.167 s), RV E (30.2-46.91 cm/s), RV A (47.1-60.7 cm/s), RV E/A (0.65-0.78 cm/s), LV E (27.0-41.4 cm/s), LV A (43.2-53.8 cm/s), and LV E/A (0.63-0.78 cm/s). Only LV A wave measurements demonstrated an intraobserver CCC >0.80. The remaining intra- and interobserver reproducibility parameters demonstrated lower CCC.Conclusions: Reference values were replicated for the fetal LV Mod-MPI and LV and RV transvalvular peak velocities between 20 and 36 + 6 weeks of gestation.

Reference ranges of filling time and systolic-to-diastolic time index of the left ventricle, right ventricle, and interventricular septum using both spectral and tissue Doppler of fetal heart between 20 and 36 + 6 weeks of gestation.

OBJECTIVE: The objective of this study is to determine the reference ranges for filling time (FT) and systolic-to-diastolic time index (SDI) of the left ventricle (LV) by using spectral Doppler, and FT' and SDI' of the LV, right ventricle, and interventricular septum (IVS) by using tissue Doppler of the fetal heart. STUDY DESIGN: This prospective and cross-sectional study included 360 low-risk singleton pregnancies between 20 and 36 + 6 weeks of gestation. The SDI/SDI' is the sum of the ejection time, isovolumic contraction time, and isovolumic relaxation time (IRT) divided by the FT. We measured FT/FT' from the beginning of the opening click of the E wave of the mitral valve to the closing click of the A wave of the mitral valve. We used regression analysis to obtain the best-fit model polynomial equation for the parameters. Additionally, we assessed intra- and inter-observer reproducibility by using concordance correlation coefficient (CCC). RESULTS: There was a weak correlation among FT LV (r = 0.31, p < 0.0001), SDI LV (r = -0.23, p < 0.0001), and gestational age (GA). Additionally, there was a very weak positive correlation among FT' RV (r = 0.09, p = 0.0001), FT' LV (r = 0.07, p < 0.0001), FT' IVS (r = 0,08, p < 0.0001), and GA. In contrast, there was a very weak negative correlation among SDI' LV (r=-0.09, p < 0.0001), SDI' IVS (r=-0.05, p < 0.0021), and GA. There was no significative correlation between SDI' RV (r=-0.06, p < 0.081) and GA. Poor/very poor intra- and inter-observer reliability was observed for all the parameters (CCC = 0.19-0.79), whereas moderate intra- and inter-observer agreement was observed for all parameters (CCC = 0.37-0.72). CONCLUSIONS: The reference ranges for FT and SDI were determined by using spectral and tissue Doppler of the fetal heart and showed a poor reproducibility.

Impact of type I and type II maternal diabetes mellitus on fetal cardiac function assessment parameters using spectral and tissue Doppler.

The aim of this study was to evaluate the impact of pregestational diabetes mellitus (DM) on fetal cardiac function two-dimensional parameters using spectral and tissue Doppler. Pregnant women between 20 and 36 + 6 weeks gestation were divided into three groups: controls, type I DM, and type II DM. The right ventricle (RV) and left ventricle (LV) annular velocity peaks were measured using spectral (E, A) and tissue (E', A', S') Doppler. The myocardial performance index was calculated as (isovolumetric contraction time [ICT] + isovolumetric relaxation time [IRT])/ejection time using tissue (MPI') and the spectral Doppler (MPI). A general linear model, with fetal heart rate as a covariant, was used to evaluate the effect of DM on the fetal heart function assessment parameters. To assess the association of type I and II DM with adverse perinatal outcomes, Fisher's exact test was used. A receiver operating characteristic curve was used to determine the best cutoff for fetal cardiac function assessment parameters to identify the neonatal composite outcomes. The sample comprised 179 pregnant women. DM had significant effect on RV and LV A peak velocities (p = 0.026 and p = 0.011, respectively). LV ICT (p < 0.001) and LV MPI (p < 0.001) were significantly affected by maternal DM. Fetuses from pregnant women with type II DM showed significantly higher LV MPI (0.492 vs. 0.459, p = 0.006) and RV S' (7.2 vs. 6.44 cm/s, p = 0.024) than controls. Fetuses from type I DM pregnant women showed increase in cardiac parameters that evaluated the LV and RV diastolic function (LV IRT' p < 0.001 and RV MPI' p = 0.044). Type I and II DM were associated with adverse perinatal outcomes: neonatal intensive care unit stay (p < 0.0001), macrosomia (p < 0.0001), hyperbilirubinemia (p < 0.0001), and hypoglycemia (p < 0.0001). The LV MPI' showed significant but moderate sensitivity in identifying the composite neonatal outcomes (AUC: 0.709, 95% CI 0.629-0.780, p < 0.001). Tissue Doppler and MPI parameters can be useful to detect subclinical cardiac dysfunction in the fetal heart of pregestational DM pregnant women.

Pulmonary Vein Stenosis in a Newborn: A Commonly Overlooked Diagnosis.

The diagnosis of primary pulmonary vein stenosis is often overlooked because its symptoms overlap lung diseases and pulmonary arterial hypertension. Its diagnosis may be difficult because the condition is progressive and associated with other defects. We present a case of pulmonary vein stenosis in a newborn with stenosis of the left-sided common pulmonary vein, diffuse hypoplasia of the superior right pulmonary vein, and atresia of the inferior right pulmonary vein.

Aneurysm of the Left Coronary Artery in Postoperative Bland-White-Garland Syndrome.

We report a case of anomalous left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome, present the challenges of performing a differential diagnosis, and discuss the treatment of the syndrome. Although ALCAPA is a rare congenital heart disease, it is one of the most common causes of myocardial ischemia in childhood and presents a diagnostic challenge. A four-year-old girl was referred to a pediatric cardiologist for evaluation of mitral valve regurgitation murmur and heart failure. The transthoracic echocardiogram demonstrated the left coronary artery (LCA) not arising from the aorta, presence of coronary collateral circulation, and moderate mitral valve regurgitation. ALCAPA was confirmed using angiotomography. The LCA was surgically reimplanted into the aorta. After 3 years of postoperative follow-up, the patient developed an LCA aneurysm. Diagnosis of cardiac ischemia in childhood remains a challenge, and careful evaluation of coronary arteries on the echocardiogram is an important tool. In this report, we present a case of ALCAPA with an uncommon postoperative outcome.

Taquicardia sinusal inapropriada: o papel da ecocardiografia fetal transvaginal: relato de caso / Inappropriate sinus tachycardia: the role of trasvaginal fetal echocardiography: case report / Taquicardia Sinusal Inapropiada: El papel de la Ecocardiografía Fetal Transvaginal ­ Relato del Caso

Introdução: A taquicardia sinusal inapropriada é rara na população em geral, tem maior prevalência em mulheres jovens e sua etiologia é desconhecida. Caracteriza-se por uma frequência cardíaca, persistentemente, elevada com uma resposta exagerada à atividade física. Objetivo: Relatar um caso raro de taquicardia sinusal inapropriada em fase precoce da gestação, enfatizando a importância da ecocardiografia fetal transvaginal. Relato de caso: Gestante encaminhada para realização de ecocardiografia transvaginal por taquicardia fetal persistente. Ao exame de nove semanas, o feto apresentava uma FC de 240 batimentos por minuto (bpm), com condução atrioventricular de um para um e sem sinais de hidropisia. Utilizados a digoxina e, posteriormente, o flecainide, ambos sem sucesso terapêutico. Iniciado sotatol com redução gradual e posterior normalização da FC fetal. O parto ocorreu a termo, concepto nasceu bem e evoluiu com taquicardia persistente. O eletrocardiograma realizado demonstrou onda P com morfologia de ritmo sinusal. As possíveis causas de taquicardia sinusal foram afastadas, confirmando assim o diagnóstico acima. Comentários: O autor reforça a importância da ecocardiografia fetal transvaginal para o diagnóstico e tratamento precoce de arritmias fetais, evitando complicações.

Introduction: The inappropriate sinus tachycardia is rare in the general population, more frequently affects young women, and its etiology is unknow. It is characterized by a persistently elevated cardiac frequency with na exaggerated response to physical activity. Objective: Report a rare case of inappropriate sinus tachycardia in the early phase of pregnancy and emphasize the importance of transvaginal fetal echocardiography. Case report: Pregnant referred due to persistente fetal tachycardia after obstretic ultrasonography. The transvaginal echocardiogram performed at 9 weeks' gestation showed a fetal heart rate of 240 beats min (bpm) with normal conduction from atria to ventricle (1:1)and no signal hydropsy. Digoxin therapy and Flecainide were used with no sucess. Sotatol use was chose when the fetal heart rate (HR) reduced to tolerable levels and then the number of heartbeats normalized at thirty six weeks gestation. The baby was born well at term and developed persistente tachycardia. The electrocardiogram performed showed P-ware morphology of sinus rhythm. Possible causes of sinus tachycardia were excluded, thus confirming the diagnosis above. Comments: The author describes the imortance of transvaginal fetal echocardiography for the diagnosis and early treatment of fetal arrhythmias avoiding complications.

Introducción: La taquicardia sinusal inapropiada es rara en la población en general, predomina mayormente en mujeres jóvenes y su etiología es desconocida. Se caracteriza por una frecuencia cardíaca, persistentemente, elevada con una respuesta exagerada a la actividad física. Objetivo: Relatar un caso raro de taquicardia sinusal inapropiada en fase precoz de la gestación, enfatizando la importancia de la ecocardiografía fetal transvaginal. Relato del caso: Gestante derivada para la realización del ecocardiograma transvaginal por taquicardia fetal persistente. En el examen de nueve semanas, el feto presentaba una FC de 240 latidos por minuto (lpm), con conducción aurículoventricular de uno para uno y sin señales de hidropesía. Se utilizó la digoxina y, posteriormente, el flecainide, ambos sin éxito terapéutico. Iniciado sotatol con reducción gradual y posterior normalización de FC fetal. El parto ocurrió a término, nació bien y evolucionó con taquicardia persistente. El electrocardiograma realizado demostró onda P con morfología de ritmo sinusal. Las posibles causas de taquicardia sinusal se eliminaron, confirmando así el diagnóstico señalado. Comentarios: El autor refuerza la importancia de la ecocardiografía fetal transvaginal para el diagnóstico y tratamiento precoz de arritmias fetales, evitando complicaciones

Fetal bradycardia and sinus node dysfunction.

The study reported here is a rare case of fetal sinus bradycardia that evolved into symptomatic bradycardia after birth, at which time the implantation of a cardiac pacemaker was indicated. Fetal echocardiography was used to diagnose the type of cardiac rhythm that caused the intra-uterine bradycardia, which enabled the initiation of the appropriate therapy approach and avoided an unnecessary interruption of the pregnancy. However, the details of the sinus bradycardia were impossible to determine in utero in this case due to sinus node dysfunction. After birth, the electrocardiogram results drew attention to a potentially unusual cause of sinus bradycardia, and enabled the diagnosis of this rare disease in this infant.

Elevated lipoprotein(a) in a newborn with thrombosis and a family history of dyslipidemia.

This paper reports a rare case of elevated lipoproteinemia(a) that evolved into thrombosis during the neonatal period. During the first days of life, the patient presented with an intracardiac thrombus, pulmonary thromboembolism and a hemorrhagic stroke. Initially, the results of the blood tests performed to screen for thrombophilic diseases were normal for the patient's age. The maternal dyslipidemia and the family's positive history of thromboembolism drew attention to an underlying, inherited, thrombophilic defect. Upon further investigation of the thrombophilia, the increase in lipoprotein(a) levels found in the mother and infant enabled the diagnosis of hyperlipoprotein(a) and the administration of appropriate therapy.

Constrição fetal do canal arterial após autouso materno de propianato de clobetasol tópico: relato de dois casos / Constriction of fetal ductus arteriosus after maternal self-use of clobetasol propianato topic: case report

A constrição prematura do ducto arterioso não é uma malformação estrutural e sim uma anormalidade funcional importante na vida fetal, podendo evoluir com insuficiência cardíaca (IC), hidropisia fetal e até óbito. Habitualmente, decorre do uso materno de drogas anti-inflamatórias (AINH) que aceleram a sensibilidade do ducto, podendo ocorrer na ausência de fatores desencadeantes identificáveis. Este estudo relata dois casos de constrição fetal ductal, após automedicação de creme de propianato de clobetasol. Objetiva-se demonstrar que essa anormalidade cardíaca fetal deve ser suspeitada pela ultrassonografia obstétrica e encaminhada para ecocardiografia fetal, exame que possibilita o diagnóstico, acompanhamento e melhora no prognóstico desses casos.

Premature ductal constrition is not a structural malformation, but may cause functional impairment during intrauterine life progressing to heart failure (CHF) and fetal hydrops witch can lead to death. Usually arises from the maternal use of drugs that speed up the sensitivity of the duct, witch may occurs in the absence of triggering factors. This study reports two cases of intrauterine ductal constriction after topic use of clobetasol. It aims to demonstrate that this fetal cardiac abnormality should be suspected by obstetric ultrasound and referred to fetal echocardiography that allows the diagnosis, monitoring and improving prognosis of these cases.

Weight and height recovery in children with Down syndrome and congenital heart disease.

OBJECTIVE: To evaluate the impact of congenital heart diseases in growth of children with Down syndrome (DS) and the weight-height recovery after surgical correction. METHODS: Retrospective study of the DS patients between 1984 and 2007. Excluding the mosaics and/or patients with associated morbidities (n=165). Calculated Z scores for weight (Zwb) and length (Zlb) at birth. Those patients submitted to surgical correction (n= 60) these scores (Zw/Zh) were evaluated before surgery and in subsequent periods to five years. Malnutrition was defined as weight/height Z-score < 2.5. Used Chi-square test to verify the relation between weight/length and age at the time of surgery and Student T test to evaluate the postoperative (PO) time of recovery (P < 0.05). RESULTS: Means Zwb (n = 162) and Zlb (n = 156) were -0.95 ± 1.27 and -1.348 ± 1.02. From the total data (n = 165), 65.5% (n = 108) presented heart disease. Those submitted to cardiac surgery (n = 60), Zw was below -2.5 in 55% (n = 33) and Zh in 60% (n = 36). After six months PO, 67.4% achieved Zw > 2.5. In one year, 85.7% achieved Zh > 2.5. Dividing this group by age in tertiles at time of surgery no difference was found. CONCLUSIONS: We concluded that malnutrition common in children with DS since birth. DS children with congenital heart and surgical indication were smaller and lighter than those without or with mild disease. PO recovery occurred in 6 months for weight and one year for height, with no difference in the age at the time of surgery.

Recuperação pôndero-estatural em crianças com síndrome de Down e cardiopatia congênita / Catch up in children with Down syndrome and congenital heart disease

OBJETIVO: Avaliar o impacto das cardiopatias congênitas em crianças no crescimento de crianças com síndrome de Down (SD) e a sua recuperação pôndero-estatural após correção cirúrgica. MÉTODOS: Estudo retrospectivo de portadores da SD, entre 1984 e 2007. Excluídos os mosaicos e/ou portadores de morbidades associadas (n=165). Calcularam-se os escores Z para peso (Zpn) e comprimento (Zen) no momento do nascimento. Nos pacientes submetidos à correção cirúrgica (n=60), os escores Zp e Ze foram calculados antes da intervenção cirúrgica e em períodos posteriores, em até cinco anos de pós-operatório (PO). Em relação à população geral, Zp e Ze < 2,5 foram significativos para déficit de crescimento. Utilizado teste do Qui-Quadrado para verificar relação entre peso/estatura e idade no momento da cirurgia e teste T de Student para avaliar o momento em que ocorreu a recuperação PO (P < 0,05). RESULTADOS: As médias de Zpn (n=162) e Zen (n=156) foram -0,95 ± 1,27 e -1,348 ± 1,02. Da amostra total (n=165), 65,5% (n=108) dos pacientes apresentavam doença cardíaca. Dentre os pacientes submetidos (n=60) à cirurgia cardíaca, Zp era inferior a -2,5 em 55% (n=33) e Ze, em 60% (n=36). Com seis meses de PO, 67,4% alcançaram Zp > 2,5. Em um ano, 85,7% atingiram Ze > 2,5. Dividindo este grupo por idade, na época da cirurgia, em tercis não ocorreu diferença. CONCLUSÕES: Observou-se déficit pôndero-estatural em relação à população geral desde o nascimento, sendo maior nas crianças com cardiopatia de indicação cirúrgica. A recuperação PO ocorreu em seis meses para o peso e em um ano para a estatura, sem diferença quanto à idade no momento cirúrgico.

OBJECTIVE: To evaluate the impact of congenital heart diseases in growth of children with Down syndrome (DS) and the weight-height recovery after surgical correction. METHODS: Retrospective study of the DS patients between 1984 and 2007. Excluding the mosaics and/or patients with associated morbidities (n=165). Calculated Z scores for weight (Zwb) and length (Zlb) at birth. Those patients submitted to surgical correction (n= 60) these scores (Zw/Zh) were evaluated before surgery and in subsequent periods to five years. Malnutrition was defined as weight/height Z-score < 2.5. Used Chi-square test to verify the relation between weight/length and age at the time of surgery and Student T test to evaluate the postoperative (PO) time of recovery (P < 0.05). RESULTS: Means Zwb (n = 162) and Zlb (n = 156) were -0.95 ± 1.27 and -1.348 ± 1.02. From the total data (n = 165), 65.5% (n = 108) presented heart disease. Those submitted to cardiac surgery (n = 60), Zw was below -2.5 in 55% (n = 33) and Zh in 60% (n = 36). After six months PO, 67.4% achieved Zw > 2.5. In one year, 85.7% achieved Zh > 2.5. Dividing this group by age in tertiles at time of surgery no difference was found. CONCLUSIONS: We concluded that malnutrition common in children with DS since birth. DS children with congenital heart and surgical indication were smaller and lighter than those without or with mild disease. PO recovery occurred in 6 months for weight and one year for height, with no difference in the age at the time of surgery.