Congenital Heart Disease and Pulmonary Hypertension (original) (raw)

An Update on Pulmonary Arterial Hypertension

The Journal for Nurse Practitioners, 2015

Pulmonary arterial hypertension (PAH) is a progressive disease that ultimately leads to right heart failure and death. PAH is defined as a mean pulmonary arterial pressure ≥ 25 mm Hg with a pulmonary capillary wedge pressure ≤ 15 mm Hg at rest. The diagnosis of PAH is one of exclusion; diagnostics include an extensive history, serology, chest radiograph, pulmonary function tests, ventilation/perfusion scan, transthoracic echocardiogram, and right heart catheterization. Treatment and care of patients with PAH can be complex. Therefore, the nurse practitioner is an integral member of the healthcare team caring for PAH patients, helping to ensure seamless care and support. Keywords pulmonary arterial hypertension; pathophysiology; diagnosis; treatment Pulmonary arterial hypertension (PAH) is a progressive disease that ultimately leads to right heart failure and death. It is hemodynamically defined as a mean pulmonary arterial pressure (mPAP) ≥ 25 mm Hg with a pulmonary capillary wedge pressure (PCWP) ≤ 15 mm Hg at rest resulting in increased pulmonary vascular resistance. 1 PAH is predominantly found in women (80%) with the mean age of diagnosis of 53 years. 1 Patients with PAH may initially report dyspnea especially on exertion along with fatigue. These symptoms can be severe 2 impairing their ability to function and their health-related quality of life (HRQOL). 2 Symptoms continue to worsen until patients are diagnosed and therapies are initiated. Diagnosis may be delayed due to the symptoms mimicking other cardiopulmonary disease resulting in worsening right ventricular modeling and increasing mortality. 3 The purpose of this review is to provide an overview and update on the pathophysiology, classification, diagnosis, and treatment of PAH.

Definitions and Diagnosis of Pulmonary Hypertension

Journal of the American College of Cardiology, 2013

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure !25 mm Hg at rest, measured during right heart catheterization. There is still insufficient evidence to add an exercise criterion to this definition. The term pulmonary arterial hypertension (PAH) describes a subpopulation of patients with PH characterized hemodynamically by the presence of pre-capillary PH including an end-expiratory pulmonary artery wedge pressure (PAWP) 15 mm Hg and a pulmonary vascular resistance >3 Wood units. Right heart catheterization remains essential for a diagnosis of PH or PAH. This procedure requires further standardization, including uniformity of the pressure transducer zero level at the midthoracic line, which is at the level of the left atrium. One of the most common problems in the diagnostic workup of patients with PH is the distinction between PAH and PH due to left heart failure with preserved ejection fraction (HFpEF). A normal PAWP does not rule out the presence of HFpEF. Volume or exercise challenge during right heart catheterization may be useful to unmask the presence of left heart disease, but both tools require further evaluation before their use in general practice can be recommended. Early diagnosis of PAH remains difficult, and screening programs in asymptomatic patients are feasible only in high-risk populations, particularly in patients with systemic sclerosis, for whom recent data suggest that a combination of clinical assessment and pulmonary function testing including diffusion capacity for carbon monoxide, biomarkers, and echocardiography has a higher predictive value than echocardiography alone. (J Am Coll Cardiol 2013;62: D42-50) ª 2013 by the American College of Cardiology Foundation Diagnosis and assessment of patients with pulmonary arterial hypertension (PAH) have been major topics at all previous world meetings on pulmonary hypertension (PH), with the last update coming from the 4th World Symposium on Pulmonary Hypertension (WSPH) held in 2008 in Dana Point, California (1). The recommendations from that conference were incorporated into the most recent international guidelines (2-4). During the 5th WSPH in 2013 in From the

Clinical features of human pulmonary hypertension

2011

printing supported by . Visit Chiesi at Stand D.30 WEDNESDAY, SEPTEMBER 28TH 2011 as the cutoff, we could distinguish IPAH from PHLHD with 97% sensitivity and 100% specificity. The area under the ROC curve was 0.99. Conclusion: Left atrial volume of 43 ml/m2 recorded by CMR may be a useful means of distinguishing between IPAH and PHLHD, avoiding the need for catheterisation. 4913 The changing picture of patients with pulmonary arterial hypertension (PAH) in France Olivier Sitbon1, Azzedine Yaïci1, Laurence Rottat1, Vincent Cottin2, Emmanuel Bergot3, Grégoire Prévot4, Virginie Gressin5, Marc Humbert1, Gérald Simonneau1. 1Centre de Référence de l’Hypertension Pulmonaire Sévère, APHP, Hôpital A. Béclère, Université Paris-Sud 11, Clamart, France; 2Service de Pneumologie, CHU, Hôpital Louis Pradel, Lyon, France; 3Service de Pneumologie, CHU, Hôpital de la Côte de Nacre, Caen, France; 4Service de Pneumologie, CHU, Hôpital Larrey, Toulouse, France; 5Actelion, Pharmaceuticals, Paris, France...

ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension

Circulation, 2009

Persistent PH of the newborn is a syndrome characterized by increased pulmonary vascular resistance, right to left shunting, and severe hypoxemia. Treatment options include inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation. Pediatric IPAH is treated similar to that in adults. A higher percentage of children are acute responders and candidates for calcium channel blockers. Physical Signs That Suggest Possible Underlying Cause or Associations of PH Central cyanosis Abnormal V/Q, intra-pulmonary shunt, hypoxemia, pulmonary-to-systemic shunt Clubbing Congenital heart disease, pulmonary venopathy Cardiac auscultatory findings, including systolic murmurs, diastolic murmurs, opening snap, and gallop Congenital or acquired heart or valvular disease Rales, dullness, or decreased breath sounds Pulmonary congestion or effusion or both Fine rales, accessory muscle use, wheezing, protracted expiration, productive cough Pulmonary parenchymal disease Obesity, kyphoscoliosis, enlarged tonsils Possible substrate for disordered ventilation Sclerodactyly, arthritis, telangiectasia, Raynaud phenomenon, rash Connective tissue disorder Peripheral venous insufficiency or obstruction Possible venous thrombosis Venous stasis ulcers Possible sickle cell disease Pulmonary vascular bruits Chronic thromboembolic PH Splenomegaly, spider angiomata, palmary erythema, icterus, caput medusa, ascites Portal hypertension PH indicates pulmonary hypertension.