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Predictors of Functional Capacity in Patients with Pulmonary Hypertension

Introduction: The 6-minute walk test (6 MWT) distance is frequently used in the prediction of pulmonary hypertension (PH) prognosis. However, potential surrogates of this measure have not been established. We aim to describe the clinical, echocardiographic, and laboratorial criteria determining the 6 MWT distance in patients with PH. Methods: In 22 consecutive PH patients, functional capacity was evaluated by the 6 MWT distance and compared with levels of neurohormonal activation biomarkers and echocardiographic parameters for right ventricular (RV) function. Results: A correlation between the 6 MWT distance and several clinical parameters was found: Borg’s pre-test classification (R=-0.46; p=0.038); WHO functional class (p=0.029). Patients with higher levels of aldosterone (R=-0.46; p=0.030), renin (R=-0.43; p=0.046) and mid-regional pro-adrenomedullin (MR-proADM, R=-0.53; p=0.009) had worse 6 MWT performance. The opposite was true for natremia levels (R=0.55; p=0.006). There was a positive correlation between the 6 MWT distance and RV global longitudinal peak systolic strain rate and early diastolic strain rate (R=0.49; p=0.038 and R=-0.55; p=0.018, respectively). On multivariate analysis, only natremia and early diastolic strain rate were predictors of the 6 MWT distance. Conclusion: The 6 MWT distance correlated with renin-angiotensin-aldosterone system activation and parameters for RV myocardial deformation. The new biomarker MR-proADM proved to be useful in the prediction of the functional capacity.

Progressão da desnervação simpática cardíaca avaliada por cintigrafia com MIBG‐I123 na polineuropatia amiloidótica familiar e o impacto da transplantação hepática

INTRODUCTION Familial amyloid polyneuropathy (FAP) is a rare disease caused by systemic deposition of amyloidogenic variants of the transthyretin (TTR) protein. The TTR-V30M mutation is caused by the substitution of valine by methionine at position 30 and mainly affects the peripheral and autonomic nervous systems. Cardiovascular manifestations are common and are due to autonomic denervation and to amyloid deposition in the heart. Cardiac sympathetic denervation detected by iodine-123 labeled metaiodobenzylguanidine (MIBG) is an important prognostic marker in TTR-V30M FAP. Liver transplantation, widely used to halt neurological involvement, appears to have a varying effect on the progression of amyloid cardiomyopathy. Its effect on the progression of cardiac denervation remains unknown. METHODS In this observational study, patients with the TTR-V30M mutation underwent annual cardiac assessment and serial MIBG imaging with quantification of the late heart-to-mediastinum (H/M) ratio. RESULTS We studied 232 patients (median age 40 years, 54.7% female, 37.9% asymptomatic at the time of inclusion) who were followed for a median of 4.5 years and underwent a total of 558 MIBG scans. During follow-up, 47 patients (20.3%) died. MIBG scintigraphy at inclusion was a strong predictor of prognosis, with the risk of death increasing by 27.8% for each one-tenth reduction in the late H/M ratio. The late H/M ratio decreased with age (0.082/year, p<0.001), but progression of cardiac denervation was so slow that annual repetition of MIBG imaging did not increase its prognostic accuracy. During follow-up, 70 symptomatic patients underwent liver transplantation. The late H/M ratio decreased by 0.19/year until transplantation but no statistically significant differences were detected after the procedure. CONCLUSIONS Cardiac denervation is common during the progression of TTR-V30M FAP and quantification of the late H/M ratio on MIBG scintigraphy is valuable for prognostic stratification of these patients. Liver transplantation stabilizes cardiac denervation, without recovery or further deterioration in cardiac MIBG uptake after the procedure.

Congenital Submitral Diverticulum

A 36-year-old Guinean woman was referred to our center for a closer examination of systolic murmur. She had no cardiovascular complaints. Echocardiographic examinations depicted a large saccular structure at the left ventricular lateral and infer-lateral walls, in the submitral region (Figure 1). The outpouching structure was connected to the left ventricle (LV) through a large neck. In diastole, blood flowed from the LV into the cavity, and in systole blood flowed from the cavity into the LV. These findings suggested that this cavity itself contracted. No other cardiac abnormalities were found. To better delineate the whole shape of the cavity, a computed tomographic scan was requested. It allowed precise measurement of the outpouching structure (9 x 7 cm), delineation of its morphology and excluded coronary artery disease (Figure 2). To confirm whether the cavity wall consisted of muscle, a cardiac magnetic resonance study was performed; cardiac magnetic resonance cine and late gadolinium enhancement images clearly demonstrated a contractile cavity wall, consisting of muscle with no fibrosis (Figure 3). A diagnosis of large congenital submitral left ventricular diverticulum was made. The patient refused surgery and is followed up at our cardiology clinic. Left ventricle diverticulum is a rare condition and it is important to differentiate it from pseudoaneurysm. While the definitive diagnosis should be based on histopathologic evaluation, a review of the literature showed that there are different clinical and radiologic criteria for distinguishing these lesions. Cardiac magnetic resonance adequately characterizes these outpouchings, allowing accurate diagnosis, a better understanding of their natural history, and can guide proper management decisions. Figure 1.

Unexpected Mass in the Left Atrium

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Estratificação prognóstica na hipertensão pulmonar: valor acrescido da abordagem multibiomarcadores

INTRODUCTION Pulmonary hypertension (PH) covers a group of conditions characterized by an increase in pulmonary vascular resistance leading to right ventricular failure. Risk stratification is crucial for adequate prognostic and therapeutic assessment. However, the accuracy of conventional parameters is limited, especially biomarkers. OBJECTIVES To determine the prognostic value of new biomarkers and their combination in a multi-biomarker approach to predict outcome in patients with PH. METHODS In this prospective cohort study, PH patients underwent clinical, echocardiographic and laboratory assessment, including quantification of serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and of the following new biomarkers: mid-regional pro-adrenomedullin (MR-proADM), copeptin, endothelin-1, mid-regional pro-atrial natriuretic peptide (MR-proANP) and soluble ST2 (sST2), the interleukin-33 receptor. The accuracy of the different parameters for predicting all-cause mortality and death or hospitalization of cardiac causes was determined. The prognostic value of a multi-biomarker score based on the tertile distribution of serum NT-proBNP, MR-proANP, renin and sST2 was compared to conventional markers. RESULTS Forty-three patients (72.1% female, age 59±15 years) were included, most of whom (65.1%) had group 1 PH. During a median follow-up of 34 months, 26% of the patients died and 35% were hospitalized for cardiac causes. Atrial and ventricular dimensions and right ventricular fractional area change were prognostic predictors. Log NT-proBNP (HR: 31.14; 95% CI: 3.12-310.7; p=0.003) and renin (HR: 1.02; 95% CI: 1.005-1.038; p=0.009) were independent predictors of mortality. MR-proANP (HR: 1.008; 95% CI 1.004-1.011; p<0.001) and sST2 (HR: 1.005; 95% CI 1.001-1.009; p=0.04) were predictors of death or hospitalization. The prognostic value of the multi-biomarker score was higher than any of the conventional parameters, and enabled identification of risk groups (the high-risk group had three-year mortality of 77.8%). CONCLUSION A multi-biomarker approach was superior for risk stratification to any single marker. A score that incorporates NT-proBNP, MR-proANP, renin and sST2 accurately identifies patients at low, intermediate and high risk.

Right ventricular hematoma

© 2016 Sociedade Portuguesa de Cardiologia. Published by Elsevier Espana, S.L.U. All rights reserved.

Left main ostial agenesis and right coronary artery occlusion: the importance of the ‘Vieussens' arterial ring’

A 76-year-old man with a medical history of hypertension, smoking, and stable angina was admitted with the diagnosis of ischaemic stroke, after 12-h of development of symptoms. During hospitalization, he frequently referred episodes of angina at rest and his electrocardiogram detected dynamic ST-depression on the lateral leads during chest pain. Echocardiography showed preserved biventricular systolic function, with no regional wall motion abnormalities. Given the high ischaemic risk an invasive coronary angiography was made. However, selective engagements of the right and …

ST-Segment–Elevation Myocardial Infarction Attributable to Left Main Coronary Artery Compression

A 44-year-old man with Eisenmenger syndrome in the setting of a subaortic ventricular septal defect under treatment with bosentan presented with angina at rest for 12 hours. He reported effort chest pain and worsened dyspnea in the previous weeks. The ECG at admission showed transient ST elevation in aVR and V1 coupled with ST depression in the other leads (Figure A), suggesting ischemia attributable to left main coronary artery (LMCA) obstruction, given the clinical context. Figure. A , Standard 12-lead ECG showing transient ST elevation in aVR and V1 coupled with ST depression in the other leads. B , Cardiac volume rendered computed tomography (CT) showing the spatial …

Takotsubo Syndrome: A Pathway through the Pituitary Disease.

Takotsubo cardiomyopathy (TTC) is characterized by reversible left ventricular apical and/or midventricular hypokinesia with unknown etiology. The clinical presentation is similar to acute myocardial infarction in the absence of significant obstructive coronary artery disease. Various predisposing factors have been related to TTC, such as acute neurological illnesses, endocrine diseases, pain, and emotional stress. We present the first description of an association between TTC cardiomyopathy and panhypopituitarism. This case reinforces the connection between the hormonal and cardiovascular systems. Furthermore, it supports the importance of a comprehensive and integrated medical history in the approach of a patient with cardiac disease, towards clinical decision-making.

An unexpected cause of myocardial infarction.

Un varon de 54 anos de edad, con antecedentes de hipertension y valvula aortica bicuspide, fue tratado con una sustitucion urgente de la valvula aortica y la raiz aortica empleando un conducto valvular compuesto (intervencion de Bentall-De Bono) a causa de una diseccion aortica aguda de tipo A. La operacion transcurrio sin incidencias, pero en el postoperatorio el paciente tuvo una elevacion del segmento ST en las derivaciones inferiores, junto con un aumento concomitante de la troponina I en suero (42 ng/ml). La exploracion fisica fue normal y la presion arterial era de 100/70 mmHg. La ecocardiografia transtoracica mostro una hipocinesia de la pared inferior, con fraccion de eyeccion del ventriculo izquierdo conservada (video 1 del material suplementario). Se realizo una angiotomografia computarizada coronaria para descartar por medios no invasivos una posible complicacion quirurgica o una diseccion arterial coronaria. Esta exploracion no mostro ninguna alteracion arterial coronaria significativa. Sin embargo, un drenaje toracico retrocardiaco (figura, punta de flecha) situado en paralelo al trayecto de la arteria coronaria derecha (figura, flecha azul) tenia su extremo distal situado de tal modo que causaba una estenosis focal importante en el origen de la arteria descendente posterior (figuras D–F, flecha amarilla; video 2 del material suplementario). Se recoloco de inmediato el drenaje, y el paciente recibio el alta a los pocos dias, y no ha sufrido nuevas complicaciones. Los drenajes de torax que se dejan sistematicamente despues de las intervenciones de cirugia cardiotoracica causan, de manera excepcional, compresion de las arterias coronarias. Que nosotros sepamos, este es el primer caso en que se describe dicha complicacion como mecanismo causal de un infarto de miocardio. Dado su campo de vision y su resolucion espacial, la angiotomografia computarizada es una tecnica de imagen ideal para visualizar las relaciones topograficas de las arterias coronarias. Esta figura se muestra a todo color solo en la version electronica del articulo.