Celia Camelo Silva

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.

A haemodynamic study of pulmonary hypertension in chronic hypersensitivity pneumonitis

Chronic hypersensitivity pneumonitis is a common fibrotic interstitial lung disease. The prevalence of pulmonary hypertension diagnosed by right heart catheterisation and its cardiopulmonary function findings in patients with chronic hypersensitivity pneumonitis are unknown. Consecutive symptomatic patients with chronic hypersensitivity pneumonitis were prospectively evaluated. All patients were submitted to right heart catheterisation, pulmonary function testing, a 6-min walk test, echocardiography, blood gas determination and N-terminal pro-brain natriuretic peptide analyses. Nonhypoxaemic patients also underwent incremental cardiopulmonary exercise testing. 50 patients underwent right heart catheterisation; 25 (50%) of these had pulmonary hypertension and 22 (44%) had a pre-capillary haemodynamic pattern. The patients with pre-capillary pulmonary hypertension had lower forced vital capacity (mean±sd 50±17% versus 69±22% predicted, p<0.01), carbon monoxide diffusing capacity (37±12% versus 47±14% predicted, p<0.01), arterial oxygen tension (median (interquartile range) 59.0 (47.8–69.3) versus 73.0 (62.2–78.5) mmHg, p<0.01) and saturation after the 6-min walk test (78±8% versus 86±7%, p<0.01). In pre-capillary pulmonary hypertension, oxygen uptake was also lower at the anaerobic threshold (41±11% versus 50±8% predicted, p=0.04) and at peak exercise (12.8±1.6 versus 15.0±2.5 mL·kg−1·min−1, p=0.02). Pre-capillary pulmonary hypertension is common in symptomatic chronic hypersensitivity pneumonitis and is related to interstitial lung disease severity. Additionally, pulmonary hypertension is more prevalent in hypoxaemic patients with impaired lung function and exercise capacity. PH is common in chronic hypersensitivity pneumonitis and is related to interstitial lung disease severity http://ow.ly/uTXXx

Usefulness of pulmonary capillary wedge pressure as a correlate of left ventricular filling pressures in pulmonary arterial hypertension

BACKGROUND Pulmonary arterial hypertension (PAH) is characterized by a pulmonary capillary wedge pressure (PCWP) of ≤15 mm Hg, given a normal left ventricular filling pressure (LVFP). However, recent studies have shown that, in PAH patients, diagnosis based on PCWP can erroneously classify a significant number of patients compared with diagnosis based on left ventricular end-diastolic pressure (LVEDP). Therefore, we sought to compare the diagnostic accuracy of end-expiratory PCWP and LVEDP measurements in patients suspected of having pulmonary hypertension (PH). METHODS We reviewed the hemodynamic data from 122 patients suspected of having PH who underwent simultaneous right- and left-side heart catheterizations at a PH referral center from 2006 to 2011. RESULTS PH was diagnosed in 105 patients, 79% of whom (n = 83) showed a pre-capillary pattern according to the LVEDP measurement. Ninety percent of patients with PCWP ≤15 mm Hg were correctly classified as having pre-capillary PH. However, 39% of patients with a PCWP >15 mm Hg had LVEDP ≤15 mm Hg and would have been erroneously diagnosed with pulmonary venous hypertension based on their PCWP measurements alone. The sensitivity and specificity was 0.89 and 0.64, respectively. A Bland-Altman analysis of the PCWP and LVEDP measurements revealed a mean bias of 0.3 mm Hg with 95% limits of agreement of -7.2 to 7.8 mm Hg. CONCLUSIONS A PCWP ≤15 mm Hg was found to be a reliable indicator of normal LVFP in pre-capillary PH patients. When measured properly and analyzed in the clinical context, PCWP is a valuable tool for accurate diagnosis of PAH.

The case for utilizing more strict quantitative Doppler echocardiographic criterions for diagnosis of subclinical rheumatic carditis.

AIM Our aim was to perform a comparative, quantitative and qualitative, analysis of valvar echocardiographic findings in patients with acute rheumatic fever, with or without clinical manifestations of carditis, as compared to healthy controls. METHODS AND RESULTS We analyzed cross-sectional Doppler echocardiographic images of 31 patients with acute rheumatic fever diagnosed according to the Jones criterions as modified in 1992. Of 31 patients, 22 presented with clinical carditis, while 9 had subclinical carditis. The patients, and a control group of 20 healthy individuals, underwent cardiac examination and echocardiographic assessment, assessing quantitative and qualitative findings of mitral and aortic valvar abnormalities. The leaflets of the mitral valve were statistically thicker in those with clinical and subclinical carditis when compared to controls (p less than 0.001). We observed a greater frequency of mitral variance, convergence of mitral flow, and aortic regurgitation for those with clinical and subclinical carditis when compared to controls (p less than 0.001, p less than 0.001 and p equal to 0.003, respectively). Patients with clinical and subclinical carditis had more quantitative and qualitative changes in the parameters than did the controls. CONCLUSION Echocardiography is a sensitive method to detect valvar abnormalities in patients with acute rheumatic fever and carditis. Additionally, by using regular standardized criterions, abnormalities that lead to a diagnosis of subclinical carditis are found in those patients with acute rheumatic fever in the apparent absence of cardiac involvement.

Avaliação clínico-cardiológica e ecocardiográfica, seqüencial, em crianças portadoras da síndrome de Marfan

OBJETIVO: Descrever a apresentacao clinica cardiologica e a evolucao temporal, estimar a incidencia de ectasia ânulo-aortica e de prolapso da valva mitral, e avaliar a tolerância e a efetividade dos betabloqueadores em criancas com sindrome de Marfan. METODOS: Foram submetidas a exame clinico e ecocardiografico seriado, durante um ano, 21 criancas com sindrome de Marfan. No ecocardiograma foram analisados: presenca de prolapso mitral, diâmetro da raiz aortica, refluxos das valvas mitral e aortica, e o crescimento dos diâmetros aorticos na vigencia de betabloqueadores. Em 11 pacientes foi possivel obter duas medidas da raiz aortica no intervalo de um ano. RESULTADOS: Durante o estudo as criancas nao apresentaram sintomas. Prolapso mitral foi encontrado em 11 (52%) criancas. Ectasia ânulo-aortica ocorreu em 16 (76%) pacientes, sendo de grau discreto em 42,8%, moderado em 9,5%, e importante em 23,8%. Um desses pacientes foi submetido com sucesso a cirurgia de Bentall DeBono. Com o uso de betabloqueador a frequencia cardiaca diminuiu 13,6% (de 85 para 73 bpm; p < 0,009), mas houve um crescimento da raiz aortica de 1,4 mm/ano (p < 0,02). Uma crianca nao pode receber betabloqueador em razao de asma bronquica, e nao foram observados efeitos colaterais significativos nas outras criancas, incluindo uma com asma bronquica. CONCLUSAO: Os resultados obtidos sugerem que, no periodo observado, as criancas permaneceram assintomaticas, o uso de betabloqueadores diminuiu significativamente a frequencia cardiaca e nao se acompanhou de efeitos adversos significativos. Ao contrario da literatura, a incidencia de ectasia ânulo-aortica foi elevada e maior do que a de prolapso valvar mitral, tendo crescimento mesmo na vigencia de uso eficaz de betabloqueador.

Uso da angiografia rotacional 3D (3D-RA) em portadores de cardiopatias congênitas: experiência de 53 casos

BACKGROUND: Advances in the diagnosis and treatment of congenital heart disease are associated to advances in imaging techniques. Anatomic images obtained by computed tomography scan, magnetic resonance imaging (MRI) and echocardiography have been useful but cannot provide accurate hemodynamic data. 3D rotational angiography (3D-RA) is a new 3D reconstruction method carried out in the cath lab that has been widely used in neurological and urological procedures. This study was aimed at evaluating the use of 3D-RA in the diagnosis and treatment of congenital heart disease METHOD: Review of catheterization results of patients with congenital heart disease referred for diagnostic assessment in which the 3D reconstruction method was employed. Philips Allure FD 10 equipment and low osmolarity contrast medium were used for angiographies RESULTS: Overall, 53 patients were reviewed and 2.2 ± 1.1 mL/kg of contrast medium were used per patient. Anatomic details not previously shown by 2D angiographies were observed in 23% of the patients. Furthermore, 3D-RA imaging was used to make treatment decisions in 49% of the patients. Exposure to radiation was not statistically different from 2D angiography. None of the patients had complications related to the method CONCLUSION: 3D-RA provided information not usually seen by conventional angiography which was useful in the treatment of selected patients with congenital heart disease. The use of 3D-RA may reduce the number of imaging tests per procedure and as a consequence, limit patient exposure to radiation and contrast media.

Signal-morphology impedance cardiography during incremental cardiopulmonary exercise testing in pulmonary arterial hypertension.

Haemodynamic responses to exercise are related to physical impairment and worse prognosis in patients with pulmonary arterial hypertension (PAH). It is clinically relevant, therefore, to investigate the practical usefulness of non‐invasive methods of monitoring exercise haemodynamics in this patient population.

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 81 mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.

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.

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

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.