Heart Diseases ; complications ; Humans ; Hypertension, Pulmonary ; etiology

Female ; Humans ; Hypertension, Portal ; complications ; Hypertension, Pulmonary ; etiology ; Middle Aged

Angioplasty ; Angioplasty, Balloon ; Chronic Disease ; Humans ; Hypertension, Pulmonary ; Pulmonary Artery ; Pulmonary Embolism/complications* ; Thrombocythemia, Essential/complications*

Cor pulmonale is defined as alteration in structure and function of the right ventricle of the heart caused by a primary disorder of the lungs. Presented are two cases of gravidocardiac patients from cor pulmonale secondary to multi-drug resistant tuberculosis. The first case is a case of a 37-year-old gravida 4 para 3 (3-0-0-3) and the second case is that of a 24-year-old pimigravid, both of which were on their third trimester with no known cardiac disease, both initially presenting with dyspnea and heart failure symptoms. The first patient was not in labor, managed conservatively and was discharged clinically improved; the latter was delivered abdominally who later succumbed to fatal arrhythmia. Presented are the strategies in management and challenges encountered in managing a pregnant cardiac patient from cor pulmonale, specifically from pulmonary tuberculosis.
Pulmonary Heart Disease ; Hypertension, Pulmonary ; Pulmonary Artery ; Heart Diseases ; Tuberculosis ; Tuberculosis, Pulmonary ; Pregnancy Complications

Child ; Humans ; Hypertension, Pulmonary ; complications ; Male ; Pulmonary Heart Disease ; complications ; Sleep Apnea, Obstructive ; complications

A case of portopulmonary hypertension characterized by repeated syncope was retrospectively analyzed. Intrahepatic or extrahepatic factor-induced portal hypertension complicated with metabolic disorder of vasoactive substances, vascular pressure, inflammation, etc. may result in systolic and diastolic dysfunction of pulmonary arteries and systemic hyperdynamic circulation, the long-term effect of which can induce vascular remodeling and consequently, pulmonary hypertension. The pathogenic process is rather insidious. Pulmonary hypertension is clinically characterized by the raised average pulmonary artery pressure, normal pulmonary capillary wedge pressure and high pulmonary vascular resistance. Currently available therapeutic approaches include drug therapy targeting on pulmonary hypertension and liver transplantation.
Blood Pressure ; Humans ; Hypertension, Portal ; complications ; diagnosis ; Hypertension, Pulmonary ; complications ; diagnosis ; Liver Transplantation ; Syncope ; complications ; diagnosis

Objective: To explore the impact of composite clinical worsening (cCW) events and its components on the prognosis of patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH). Methods: This is a retrospective study. Patients who were diagnosed with CHD-PAH in Beijing Anzhen Hospital between January 2007 and July 2018, were included, and their baseline clinical data including demographic, clinical manifestations and New York Heart Association (NYHA) classification were collected retrospectively. All-cause deaths and clinical worsening events were recorded, which included syncope, PAH related hospitalization, NYHA classification deterioration and ≥ 2 PAH related clinical symptoms (dyspnea, hemoptysis, edema, chest pain, palpitations, cyanosis) appearance/progress. Three kinds of cCW events were defined: cCW1 (included PAH related hospitalization, NYHA classification deterioration), cCW2 (increased syncope on the basis of cCW1) and cCW3 (increased ≥ 2 PAH related clinical symptoms appearance/progress on the basis of cCW2). The Kaplan-Meier survival curve was used to analyze the long-term survival of the included patients. Univariate and multivariate Cox regression models were used to evaluate the impact of cCW events and their components on the risk of all-cause mortality. Results: A total of 525 patients with CHD-PAH were included in this study. The median age at diagnosis was 20.7 (11.2, 30.3) years. There were 43.8% children (<18 years), and 68.8% female patients. There were 431 patients (82.1%) with NYHA classification II. A total of 180 patients had PAH symptoms at diagnosis. The median follow-up time was 4.5 (2.6, 6.7) years. Forty-seven patients (9.0%) died during the follow-up period. Survival rates at 1, 5 and 10 years after diagnosis of PAH were 98.0%, 89.9% and 84.4%, respectively. Cox multivariate analysis showed that NYHA classification deterioration (HR=3.901, 95%CI 1.863-8.169, P<0.001), ≥2 PAH symptoms appearance/progress (HR=4.458, 95%CI 1.870-10.625, P<0.001), PAH-related hospitalization (HR=4.058, 95%CI 1.851-8.896, P<0.001) and syncope (HR=11.313, 95%CI 4.860-26.332, P<0.001) were independent predictors of increased risk of death. All 3 kinds of cCW events were significantly associated with the significantly increased risk of death, and cCW2 was highly predictive to increased risk of death (HR=15.476, 95%CI 4.346-37.576, P<0.001). Conclusions: The overall long-term prognosis of CHD-PAH patients in this study is relatively good. cCW events and its components (NYHA classification deterioration, ≥2 PAH symptoms occurrence/worsening, PAH-related hospitalization and syncope) have adverse influence on all-cause death in this patient cohort.
Child ; Familial Primary Pulmonary Hypertension/complications* ; Female ; Heart Defects, Congenital/complications* ; Humans ; Hypertension, Pulmonary/complications* ; Male ; Pulmonary Arterial Hypertension ; Retrospective Studies

We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities.
Adult ; Female ; Focal Nodular Hyperplasia/*radiography ; Humans ; Hypertension, Portal/*complications ; Hypertension, Pulmonary/*complications ; Portal Vein/*abnormalities

Heart Defects, Congenital ; complications ; surgery ; Humans ; Hypertension, Pulmonary ; etiology ; surgery

Humans ; Hypertension, Pulmonary ; complications ; physiopathology ; Ventricular Dysfunction, Right ; etiology