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Introduction: Pulmonary hypertension (PH) due to left-sided heart disease (Group 2 PH) is most commonly due to passive backward transmission of elevated left-sided heart pressures. A subgroup of these patients develop pulmonary artery remodeling, resulting in systolic and mean pulmonary artery pressures that are 'reactive' or 'out of proportion' to the elevated left-sided heart pressures. These patients have historically been identified by a transpulmonary gradient (TPG) > 12 mmHg. However, since diastolic pulmonary artery pressures are less susceptible to changes in pulmonary blood flow, a diastolic pulmonary gradient (DPG) ⩾ 7 mmHg may be a superior indicator of worse clinical function and prognosis., Methods: Based on measurements from a right-heart catheterization (RHC) database (Baylor College of Medicine, Houston, TX, USA), comparisons of several clinical parameters were made among three Group 2 PH patients: normal TPG and DPG (Group 2A), elevated TPG and normal DPG (Group 2B), and elevated TPG and DPG (Group 2C)., Results: Among 173 RHCs, 39 were found to have Group 2 PH. Among these 39 cases, 10 (32%), 13 (32%), and 16 (36%) cases were categorized into Groups 2A, 2B, and 2C, respectively. Group 2C patients had significantly worse 6-min walk distance (308 ± 69 m) than Group 2A (425 ± 81 m) and Group 2B (350 ± 103 m) (p = 0.038). There was no significant difference in cardiac output, cardiac index, and right atrial pressure among the three subgroups., Discussion: Among patients with Group 2 PH, elevated DPG proved to be a more specific indicator of worse functional status than the historically used TPG. International guidelines and protocols for performing RHC for the assessment and diagnosis of PH should include the measurement of DPG., (© The Author(s), 2015.)

Background: Pericardial effusion is an independent predictor of mortality in patients with pulmonary arterial hypertension (PAH). However, the management and outcomes of patients with pulmonary hypertension (PH) and pericardial effusion are not well described., Methods: A retrospective, observational study was conducted at Baylor College of Medicine and The Methodist Hospital by screening all patients admitted between 1 June 2005 and 1 June 2010 with the International Classification of Diseases, ninth revision codes for PH and pericardial effusion. A total of 138 patients were identified, and 103 patients were excluded on the basis of valvular heart disease, recent surgery or end-stage renal disease. Thirty-five patients with PH diagnosed by a historical right heart catheterization or echocardiography and with documented pericardial effusion were included in this analysis. Demographic, hemodynamic, laboratory and survival data were collected., Results: The mean age was 49.5±36 years (mean ± standard deviation), 31 of 35 patients were women (93%) and pulmonary artery systolic pressure was 77 ± 19 mmHg. Mean follow-up period was 20.5 ± 12.9 months. Fifteen patients had PAH associated with connective tissue disease (50%). The majority of the patients (87%) with pericardial effusion were managed conservatively. Four patients (13%) who were hemodynamically unstable underwent pericardial window placement. One of them was started on epoprostenol, and two patients had the doses of PAH-specific medications uptitrated. Three of four pericardial window patients survived to the conclusion of the follow-up period. The overall survival in our cohort was 60%, with three patients lost to follow-up., Conclusions: Connective tissue disease associated PAH and female sex were predominant in our cohort of patients with pericardial effusion. Seventy-five percent of patients who were treated with pericardial window for hemodynamically unstable pericardial effusion survived until the end of the study period. Pericardial window may be a therapeutic option in patients with unstable PH with pericardial effusion. Further studies are needed to determine the optimal treatment strategy for such patients.

Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling that limits the ability of the pulmonary vascular bed to withstand the physiological changes of pregnancy. Historically, pregnancy in PAH carries a high risk to the parturient. Normal pulmonary vasculature can withstand the hemodynamic and physiological changes associated with pregnancy without the development of respiratory symptomatology. However, in the presence of pulmonary vascular remodeling the capacity to handle these changes is compromised. During pregnancy, increase in cardiac output from the increased intravascular volume can lead to right heart failure. Therefore, all patients with PAH of childbearing potential should receive preconception counseling and be advised to use two methods of contraception. Patients with PAH should be advised against continuing pregnancy if they do become pregnant. According to the literature, deterioration in pregnancy mainly occurs in the second trimester and early in the third trimester; immediately postpartum is the most critical time for patients with PAH. In this review, we will discuss the recent advances in the management of parturient patients with PAH.

A 49-year-old man with Ehlers-Danlos syndrome developed acute respiratory failure requiring mechanical ventilation. Chest computed tomography demonstrated giant right bulla extending into the contralateral hemithorax with mediastinal shift. Surgical bullectomy with pleurodesis relieved tension effects and allowed weaning.