Pulmonary venous flow index as a predictor of pulmonary vascular resistance variability in congenital heart disease with increased pulmonary flow: a comparative study before and after oxygen inhalation.

AIMS: There is no definitive and reliable echocardiographic method for estimating the pulmonary vascular resistance (PVR) to differentiate persistent vascular disease from dynamic pulmonary hypertension. The aim of this study was to analyze the relationship between the pulmonary venous blood flow velocity-time integral (VTIpv) and PVR. METHODS AND RESULTS: Eighteen patients (10 females; 4 months to 22 years of age) with congenital heart disease and left to right shunt were studied. They underwent complete cardiac catheterization, including measurements of the PVR and Qp:Qs ratio, before and after 100% oxygen inhalation. Simultaneous left inferior pulmonary venous flow VTIpv was obtained by Doppler echocardiography. The PVR decreased significantly from 5.0 ± 2.6 W to 2.8 ± 2.2 W (P = 0.0001) with a significant increase in the Qp:Qs ratio, from 3.2 ± 1.4 to 4.9 ± 2.4 (P = 0.0008), and the VTIpv increased significantly from 22.6 ± 4.7 cm to 28.1 ± 6.2 cm (P = 0.0002) after 100% oxygen inhalation. VTIpv correlated well with the PVR and Qp:Qs ratio (r = -0.74 and 0.72, respectively). Diagnostic indexes indicated a sensitivity of 86%, specificity of 75%, accuracy of 83%, a positive predictive value of 92% and a negative predictive value of 60%. CONCLUSION: The VTIpv correlated well with the PVR. The measurement of this index before and after oxygen inhalation may become a useful noninvasive test for differentiating persistent vascular disease from dynamic and flow-related pulmonary hypertension.

Early Six-Minute Walk Test May Predict Midterm Outcomes Following Coronary Artery Bypass Grafting.

OBJECTIVE: This study aims to investigate the ability of the six-minute walk distance (6MWD) as a prognostic marker for midterm clinical outcomes three months after coronary artery bypass grafting (CABG), to identify possible predictors of fall in 6MWD in the early postoperative period, and to establish the percentage fall in early postoperative 6MWD, considering the preoperative baseline as 100%. METHODS: A prospective cohort of patients undergoing elective CABG were included. The percentage fall in 6MWD was assessed by the difference between preoperative and postoperative day (POD) five. Clinical outcomes were evaluated three months after hospital discharge. RESULTS: There was a significant decrease in 6MWD on POD5 compared with preoperative baseline values (percentage fall of 32.5±16.5%, P<0.0001). Linear regression analysis showed an independent association of the percentage fall of 6MWD with cardiopulmonary bypass (CPB) and preoperative inspiratory muscle strength. Receiver operating characteristic curve analysis revealed that the best cutoff value of percentage fall in 6MWD to predict poorer clinical outcomes at three months was 34.6% (area under the curve = 0.82, sensitivity = 78.95%, specificity = 76.19%, P=0.0001). CONCLUSION: This study indicates that a cutoff value of 34.6% in percentage fall of 6MWD on POD5 was able to predict poorer clinical outcomes at three months of follow-up after CABG. Use of CPB and preoperative inspiratory muscle strength were independent predictors of percentage fall of 6MWD in the postoperative period. These findings further support the clinical application of 6MWD and propose an inpatient preventive strategy to guide clinical management over time.

Risk Factors for Mortality in Children with Congenital Heart Disease Delivered at a Brazilian Tertiary Center.

OBJECTIVE: This study aims to investigate the incidence of postnatal diagnosis of congenital heart disease (CHD) and the predictive factors for hospital mortality. METHODS: This retrospective cohort study was conducted at a Brazilian tertiary center, and data were collected from medical records with inclusion criteria defined as any newborn with CHD diagnosed in the postnatal period delivered between 2015 and 2017. Univariate and multivariate analyses were performed to determine the potential risk factors for mortality. RESULTS: During the 3-year period, 119 (5.3%) children of the 2215 children delivered at our institution were diagnosed with CHD. We considered birth weight (P=0.005), 1st min Apgar score (P=0.001), and CHD complexity (P=0.013) as independent risk factors for in-hospital mortality. The most common CHD was ventricular septal defect. Indeed, 60.5% cases were considered as "complex" or "significant" CHDs. Heart surgeries were performed on 38.9% children, 15 of whom had "complex" or "significant" CHD. A mortality rate of 42% was observed in this cohort, with 28% occurring within the initial 24 h after delivery and 38% occurring in patients admitted for heart surgery. CONCLUSION: The postnatal incidence of CHD at our service was 5.3%. Low 1st min Apgar score, low birth weight, and CHD complexity were the independent factors that affected the hospital outcome.

Early Six-Minute Walk Test May Predict Midterm Outcomes Following Coronary Artery Bypass Grafting

ABSTRACT Objective: This study aims to investigate the ability of the six-minute walk distance (6MWD) as a prognostic marker for midterm clinical outcomes three months after coronary artery bypass grafting (CABG), to identify possible predictors of fall in 6MWD in the early postoperative period, and to establish the percentage fall in early postoperative 6MWD, considering the preoperative baseline as 100%. Methods: A prospective cohort of patients undergoing elective CABG were included. The percentage fall in 6MWD was assessed by the difference between preoperative and postoperative day (POD) five. Clinical outcomes were evaluated three months after hospital discharge. Results: There was a significant decrease in 6MWD on POD5 compared with preoperative baseline values (percentage fall of 32.5±16.5%, P<0.0001). Linear regression analysis showed an independent association of the percentage fall of 6MWD with cardiopulmonary bypass (CPB) and preoperative inspiratory muscle strength. Receiver operating characteristic curve analysis revealed that the best cutoff value of percentage fall in 6MWD to predict poorer clinical outcomes at three months was 34.6% (area under the curve = 0.82, sensitivity = 78.95%, specificity = 76.19%, P=0.0001). Conclusion: This study indicates that a cutoff value of 34.6% in percentage fall of 6MWD on POD5 was able to predict poorer clinical outcomes at three months of follow-up after CABG. Use of CPB and preoperative inspiratory muscle strength were independent predictors of percentage fall of 6MWD in the postoperative period. These findings further support the clinical application of 6MWD and propose an inpatient preventive strategy to guide clinical management over time.

Contrasting cardiopulmonary responses to incremental exercise in patients with schistosomiasis-associated and idiopathic pulmonary arterial hypertension with similar resting hemodynamic impairment.

It has been reported that schistosomiasis-associated PAH (Sch-PAH) has a more benign clinical course compared with idiopathic PAH (IPAH). We therefore hypothesized that Sch-PAH subjects would present with less impaired cardiopulmonary and metabolic responses to exercise than IPAH patients, even with similar resting pulmonary hemodynamic abnormalities. The aim of this study was to contrast physiologic responses to incremental exercise on cycle ergometer between subjects with Sch-PAH and IPAH. We performed incremental cardiopulmonary exercise tests (CPET) in subjects newly diagnosed with IPAH (n = 9) and Sch-PAH (n = 8), within 1 month of the hemodynamic study and before the initiation of specific therapy for PAH. There were no significant between-group differences in cardiac index, pulmonary vascular resistance or mean pulmonary artery pressure. However, mean peak oxygen uptake (VO2) was greater in Sch-PAH than IPAH patients (75.5±21.4 vs 54.1±16.1% predicted, p = 0.016), as well as the ratio of increase in VO2 to work rate (8.2±1.0 vs 6.8±1.8 mL/min/W, p = 0.03). Additionally, the slope of the ventilatory response as a function of CO2 output was lower in Sch-PAH (40.3±3.9 vs 55.6±19.8; p = 0.04), and the heart rate response for a given change in VO2 was also diminished in Sch-PAH compared to IPAH (80.1±20.6 vs 123.0±39.2 beats/L/min; p = 0.02). In conclusion, Sch-PAH patients had less impaired physiological responses to exercise than IPAH subjects with similar resting hemodynamic dysfunction. Our data suggest a more preserved cardiopulmonary response to exercise in Sch-PAH which might be related to its better clinical course compared to IPAH.

Risk factors for mortality in children with congenital heart disease delivered at a brazilian tertiary center

Abstract Objective: This study aims to investigate the incidence of postnatal diagnosis of congenital heart disease (CHD) and the predictive factors for hospital mortality. Methods: This retrospective cohort study was conducted at a Brazilian tertiary center, and data were collected from medical records with inclusion criteria defined as any newborn with CHD diagnosed in the postnatal period delivered between 2015 and 2017. Univariate and multivariate analyses were performed to determine the potential risk factors for mortality. Results: During the 3-year period, 119 (5.3%) children of the 2215 children delivered at our institution were diagnosed with CHD. We considered birth weight (P=0.005), 1st min Apgar score (P=0.001), and CHD complexity (P=0.013) as independent risk factors for in-hospital mortality. The most common CHD was ventricular septal defect. Indeed, 60.5% cases were considered as "complex" or "significant" CHDs. Heart surgeries were performed on 38.9% children, 15 of whom had "complex" or "significant" CHD. A mortality rate of 42% was observed in this cohort, with 28% occurring within the initial 24 h after delivery and 38% occurring in patients admitted for heart surgery. Conclusion: The postnatal incidence of CHD at our service was 5.3%. Low 1st min Apgar score, low birth weight, and CHD complexity were the independent factors that affected the hospital outcome.

Anomalias vasculares arteriais múltiplas em recém-nascido. Diagnóstico ecocardiográfico e angiográfico / Multiple arterial anomalies in the newborn infant. Echocardiographic and angiographic diagnosis

Multiple arterial anomalies characterized by tortuosity and rolling of the pulmonary arteries and aorta were diagnosed on echocardiography in an asymptomatic newborn infant with a phenotype suggesting Ehlers-Danlos syndrome. These changes were later confirmed on angiography, which also showed peripheral vascular abnormalities. The electrocardiogram showed a probable hemiblock of the left anterosuperior branch, and the chest x-ray showed an excavated pulmonary trunk with normal pulmonary flow.

Associaçäo de atresia pulmonar com septo interventricular íntegro e estenose valvar aórtica: diagnóstico pré-natal / Association of pulmonary atresia with intact ventricular septum and aortic valve stenosis: prenatal diagnosis

A rare association of pulmonary atresia with an intact septum was diagnosed through echocardiography in a fetus 32 weeks of gestational age. The diagnosis was later confirmed by echocardiography of the newborn infant and further on autopsy. The aortic valve was bicuspid with a pressure gradient of 81mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.

Fístula de artéria coronária direita com insuficiência cardíaca congestiva em neonato: diagnóstico ecodopplercardiográfico e tratamento por oclusäo com baläo descartável / Right coronary artery fistula with congestive heart failure in the neonate: Doppler echocardiographic diagnosis and closure with detachable balloon

We report here a case of coronary artery fistula in a neonate with clinical signs of heart failure. The electrocardiogram showed signs of left ventricular hypertrophy and diffuse alterations in ventricular repolarization. Chest X-ray showed an enlargement of the cardiac silhouette with an increase in pulmonary flow. After echocardiographic diagnosis and angiographic confirmation, closure of the fistulous trajectory was performed with a detachable balloon with an early and late successful outcome.

Drenagem venosa anômala de pulmão esquerdo. Achados clínicos e cirúrgicos e dificuldades diagnósticas / Anomalous pulmonary venous drainage of the left lung. clinical and surgical findings and dingnostic problems

PURPOSE: To analyze clinical and surgical aspects of patients with anomalous left pulmonary venous drainage (ALPVD). METHODS: Seven patients, 3 males, with ages ranging from 18 months to 29 years were retrospectively studied, by analyzing the symptoms, electrocardiograms, chest X-ray, echocardiograms, angiography and surgical technique. RESULTS: All patients, but one, were symptomatic being the effort dyspnea the most prevalent symptom. Electrocardiogram showed incomplete right bundle branch block in all but one case. Chest X-ray showed prominent pulmonic vessels (6 patients), different degrees of cardiomegaly (6) and a left superior vena cava like image (3). Echocardiography correctly depicted the anomaly in 4 cases. The ALPVD was lobar in 4 cases and total in 3. In 3 patients there was pulmonary hypertension and in one venous drainage obstruction (8mmHg gradient). Six patients were operated on, 3 through a sternotomy approach with cardiopulmonary bypass and other 3 through a left thoracotomy without cardiopulmonary bypass. CONCLUSION: ALPVD has varying clinical repercussion and its clinical picture resembles atrial septal defect. Some cases may present diagnostic difficulties and echocardiograms may result in false negative findings. Cardiac catheterization is important to obtain detailed anatomic diagnosis, to evaluate the degree of pulmonary hypertension and to rule out venous obstruction. Left thoracotomy without extracorporeal circulation is indicated in ALPVD without associated defects.

Objetivo -Analisar aspectos clínicos e cirúrgicos de portadores de drenagem anômala de veias pulmonares esquerdas (DAVPE). Métodos - Estudaram-se, retrospectivamente, 7portadores de DAVPE, sendo 3 do sexo masculino, com idades variando de 18 meses a 29 anos, analisando-se as variáveis: sintomas, padrão eletrocardiográfico, achados radiológicos, ecocardiográficos, angiográficos e técnicas operatórias. Resultados - Todos os pacientes, exceto um, eram sintomáticos sendo a queixa predominante dispnéia aos esforços. Seis apresentavam bloqueio incompleto do ramo direito. Os achados radiológicos encontrados foram: aumento da trama vascularpulmonar (6 casos), cardiomegalia (6) e imagem tipo "cova esquerda" (3). 0 ecocardiograma demonstrou corretamente a anomalia em 4 casos. No estudo hemodinâmico a drenagem anômala era restrita ao lobo superior em 4 casos e em 3 envolvia todo o pulmão esquerdo. Em 3 havia hipertensão arterial pulmonar e em 1, foi observada obstrução da drenagem venosa com gradiente pressórico de 8mmHg. Seis pacientes foram operados até o momento, sem complicações, 3, através de esternotomia e utilização de circulação extracorpórea (CEC) para correção de defeitos associados, e 3, através de toracotomia lateral esquerda sem emprego de CEC. Conclusão -DAVPE tem repercussão clínica variáveL assemelhando-se em seu quadro clínico, achados eletrocardiográficos e radiológicos com comunicação interatrial. Alguns casos podem trazer dificuldades diagnósticas e a avaliação ecocardiográfica pode resultar em diagnósticos falso negativos. O estudo hemodinâmico é importante para diagnóstico anatômico, avaliação do grau de repercussão no território pulmonar e para descartar obstrução à drenagem venosa. Toracotomia lateral esquerda sem CEC está indicada para os casos deDAVPE sem defeitos associados