Prognostic stratification in pulmonary hypertension: A multi-biomarker approach. / 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.

Reversible catecholamine-induced cardiomyopathy due to pheochromocytoma: case report.

Pheochromocytoma is a tumor originating from chromaffin tissue. It commonly presents with symptoms and signs of catecholamine excess, such as hypertension, tachycardia, headache and sweating. Cardiovascular manifestations include catecholamine-induced cardiomyopathy, which may present as severe left ventricular dysfunction and congestive heart failure. We report a case of pheochromocytoma which was diagnosed following investigation of dilated cardiomyopathy. We highlight the dramatic symptomatic improvement and reversal of cardiomyopathy, with recovery of left ventricular function after treatment.

Pulmonary hypertension in Portugal: first data from a nationwide registry.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical. METHODS: We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH). RESULTS: Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min(-1)·m(-2)); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, P = 0.015). CONCLUSIONS: We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.

Carcinoid heart disease: outcome after balloon pulmonary valvuloplasty.

Carcinoid heart disease typically presents with pulmonary stenosis and tricuspid regurgitation. Management is intended for symptomatic relief, and valvular intervention is indicated in refractory heart failure. Balloon valvuloplasty is an option for patients not suitable for surgery. We report the case of a patient with a carcinoid tumour, who developed postoperative refractory hypoxemia. Transthoracic echocardiogram revealed carcinoid pulmonary and tricuspid valve disease, with severe pulmonary stenosis. Balloon valvuloplasty was performed with major clinical improvement.

[Biomarkers of functional class in systolic heart failure: the relevance of copeptin]. / Biomarcadores da classe funcional na insuficiência cardíaca sistólica. Relevância da copeptina.

INTRODUCTION: Functional class is an important predictor of prognosis in chronic heart failure (CHF). However, it is often subjective and poorly reproducible. OBJECTIVE: We sought to identify diagnostic markers of high functional class. METHODS: We prospectively studied 37 patients with symptomatic CHF and ejection fraction<40%. The study protocol included clinical evaluation, echocardiography (M-mode, 2D, Doppler and tissue Doppler) and laboratory tests including copeptin, vasopressin and NT-proBNP. We compared patients in NYHA class II with those in NYHA class>II. Overall mortality was assessed at 18 months. RESULTS: Mortality was higher in the more advanced symptomatic stages (p<0.05). Patients in NYHA class>II had higher creatinine, copeptin and NT-proBNP levels (p<0.05). E/E', E-septum distance, pulmonary artery systolic pressure (PASP) and inferior vena cava (IVC) dimensions were also significantly greater (p<0.05). The biomarkers copeptin (area under the curve [AUC]=0.76, p<0.01) and NT-proBNP (AUC=0.81, p<0.01) and the echocardiographic parameters PASP (AUC=0.88, p<0.01) and IVC inspiratory diameter (AUC=0.91, p<0.01) showed the best performance for diagnosis of functional class>II. In multivariate regression analysis only PASP and serum creatinine were independent predictors of NYHA functional class>II. CONCLUSION: Copeptin and NT-proBNP have high sensitivity and specificity in the diagnosis of functional classes with prognostic impact and may be useful in defining a standardized functional classification. The structural and hemodynamic echocardiographic changes associated with NYHA class>II were left ventricular filling pressure, PASP and central venous pressure.

[Takotsubo syndrome or acute myocarditis? The role of cardiac magnetic resonance imaging]. / Síndroma de takotsubo ou miocardite aguda? O papel da ressonância magnética cardíaca.

Acute myocarditis is often misdiagnosed, and its evolution is not always benign; correct and prompt diagnosis is therefore essential. We report the case of a 62-year-old woman with chest pain after a stressful event and ST-segment elevation on the electrocardiogram, in whom urgent cardiac catheterization showed normal coronary arteries and left ventricular apical ballooning, suggesting takotsubo syndrome. However, cardiac magnetic resonance imaging showed lesions typical of acute myocarditis, thus suggesting this diagnosis. We highlight the diagnostic difficulty in patients with chest pain, elevated troponin and normal coronary arteries, and the key role of cardiac magnetic resonance in differential diagnosis.

[Novel mutation in long QT syndrome in a patient with prior diagnosis of epilepsy]. / Nova mutação na síndroma de QT Longo em doente com diagnóstico prévio de epilepsia.

Congenital long QT syndrome (LQTS) can present as syncope or seizures, secondary to polymorphic ventricular tachycardia, mimicking a primary seizure disorder. In patients treated with an implantable cardioverter-defibrillator (ICD), the recurrence of arrhythmias with subsequent frequent therapeutic shocks may cause adverse reactions, which can be psychogenic. We report the case of a 22-year-old woman with syncope and seizures who was diagnosed in childhood as epileptic and in whom LQTS was diagnosed only in adulthood. Beta-blocker therapy failed and an ICD was implanted. However, as arrhythmias persisted, left cardiac sympathetic denervation was performed. After surgery, three-month follow-up showed a significant reduction in arrhythmias. The genetic study identified a heterozygous mutation, c.1817 C>T p.S606F, on the KCNH2 gene that has not previously been reported in the literature. We also report the rare occurrence of an electrical storm in the course of H1N1 infection. This case illustrates the difficulties in the diagnosis and treatment of LQTS. The possibility of a common genetic basis for arrhythmic diseases and epilepsy is discussed.

Ecocardiografía tridimensional en la cardiopatía carcinoide / Three-dimensional echocardiography in carcinoid heart disease

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