Clinical and therapeutical follow up of HIV associated pulmonary hypertension: prospective study of 10 patients

OBJECTIVE: To determine the clinical course and prognosis of pulmonary hypertension (PH) in HIV-infected patients in comparison with a group of PH patients without HIV infection. The secondary objective was to ascertain whether more powerful antiretroviral treatments (highly active antiretroviral therapy) could modify the course of PH in HIV-infected patients. DESIGN: Patients with PH and HIV (HIV-PH, group 1) and patients without HIV (PPH, group 2) were prospectively followed. SETTING: A tertiary care institution. PATIENTS: Group 1 included 10 patients, and group 2 included 25 patients. INTERVENTIONS: In group 1, HIV infection was staged according to Centers for Disease Control and Prevention (CDC) classification when patients entered the study, and was re-staged every fourth month. In both groups, PH functional classes and right heart catheterization (RHC) were determined at baseline. RESULTS: In group 1, one of 10 patients was assigned to New York Heart Association (NYHA) class II, seven patients to NYHA class III, and two patients to NYHA class IV. CDC stages ranged from A1 (three patients) to C3 (one patient). No patient showed progression of HIV disease during follow-up. By May 2001, six patients had died. The median survival by the time of RHC was 15.1 months. Causes of death were heart failure in three cases, sepsis in two, and suicide in one case. In seven patients, epoprostenol was started; three patients survived and four died. The cause of death was heart failure in one patient, suicide in one, non-catheter-related sepsis in one patient and catheter-related sepsis in the last patient. In group 2, 11 patients out of 25 were assigned to NYHA class II, 11 patients to NYHA class III, and three patients to NYHA class IV. RHC was not statistically different in the two groups. By May 2001, nine of 25 patients died and one underwent a double-lung transplant. The median survival from the time of RHC was 6.86 months. Cumulative survival rates by RHC were not statistically different (hazard ratio close to 1). CONCLUSIONS: In HIV-infected patients, the onset of PH adversely affects the prognosis at any stage of infection. Clinically adverse progression of PH is not correlated with HIV initial stage and evolution. Moreover, prognosis in patients with sporadic or familial PPH and in patients with HIV-PH with similar RHC is so similar as to strengthen the concept that pulmonary vascular disease overshadows the overall clinical problem in HIV-infected patients

Holter monitoring in AL amyloidosis: prognostic implications.

The heart is involved in more than one third of patients with primary (AL) amyloidosis at diagnosis and it is by far the most common cause of death. Rhythm and conduction abnormalities generally represent the terminal event. The aims of this study were to determine the spectrum of Holter abnormalities found in AL amyloidosis and to assess their prognostic significance, particularly in relation to sudden death. Fifty-one patients with AL amyloidosis were included, and all of them had a complete history, physical examination, two-dimensional echocardiography, and 24-hour Holter monitoring. Fifty-five percent of these patients had echographic signs of heart involvement and 23\% had heart failure. Complex ventricular arrhythmias were found in 57\% of patients, couplets in 29\%, and nonsustained ventricular tachycardia in 18\%. Overall median survival was 23.4 months. Congestive heart failure, echocardiographic abnormalities, and Holter abnormalities adversely affected survival. The multivariate analysis demonstrated that interventricular septum thickness and couplets were independent predictors of survival. The presence of couplets correlated with sudden death. Holter monitoring may contribute to assessing the prognosis of patients with AL amyloidosis

Bronchogenic cyst. Unexpected finding in a large aneurysm of the pars membranacea septi

Intracardiac bronchogenic cysts are rare, with only 5 cases reported to date: 2 in the atrial septum, 1 in the left atrium, and 2 in the right ventricle. They are identified by asymptomatic occasional findings or nonspecific symptoms, mostly in the second to fourth decades. Bronchogenic cysts are believed to represent a localized portion of the tracheobronchial tree that separates from the normal airways during the branching process and does not undergo further development. They develop between the 26th and the 40th day of intrauterine life, during the most active period of airway development. The timing of the abnormal budding may determine the location: earlier in the mediastinum and later within lung tissue, which are the 2 most common locations. Intracardiac location can be explained with a more precocious abnormal budding, approximately 21 days after fertilization, when the cardiac primordial tube is near the foregut or primitive tracheobronchial tree. Other noncardiac, atypical locations are cutaneous, retroperitoneal, cervical, intradiaphragmatic, intrapericardial, intraspinal, and intrapleural. We report a case of a bronchogenic cyst located in the wall of a large aneurysm of the pars membranacea septi triggering premature ventricular complex (PVC) and sinus tachycardia

Usefulness and limits of transthoracic echocardiography in the evaluation of patients with primary and chronic thromboembolic pulmoanry hypertension

The aim of the study was to evaluate the potential usefulness of transthoracic echocardiography in differentiating patients with primary or chronic thromboembolic pulmonary hypertension and to define the capability of echocardiography to assess right-heart performance in such patients. Right-heart catheterization and ultrasound examination were performed in 111 patients with chronic thromboembolic pulmonary hypertension and in 31 patients with primary pulmonary hypertension. All echocardiographic and Doppler parameters were similar in primary and chronic thromboembolic pulmonary hypertension. A significant correlation was found between the tricuspid annular plane systolic excursion and the right ventricular fractional area change and thermodilution-derived right ventricular ejection fraction (P <.001 for both). Furthermore, different patterns of the pulsed Doppler flow velocity curve into the superior vena cava were associated with different right-heart hemodynamic profiles. In conclusion, in patients with chronic pulmonary hypertension transthoracic echocardiography portends meaningful information on the capability of the right heart to confront the increased afterload but it does not permit etiologic differentiation