Pulmonary hypertension is defined as a mean pulmonary artery pressure (mPAP) ≥25 mmHg, with Pulmonary Capillary Wedge Pressure ≤ 15 mmHg, measured by cardiac catheterization. The etiology of PH has a varied spectrum extending right from Drugs, toxins and portal hypertension to HIV, Collagen Vascular Diseases and Persistent Pulmonary Hypertension of Newborn, etc.
The estimation of disease prevalence has been nearly impossible owing to the geographic distribution and economic diversity, along with significant regional variations in human development and healthcare infrastructure. A large number of patients with PH never reach the health centers capable of diagnosing the disease condition correctly.
Advance pulmonary vascular disease as a result of uncorrected CHD is a major health challenge in the developing world.
PH exists as a major component of many forms of cardiac and pulmonary disease. While breathlessness is the most common feature of PH, patients often also present with chest pain, syncope, fatigue, weakness and abdominal distension. The precordial signs include a right ventricular lift, accentuated pulmonary component of S2, a pansystolic murmur of Tricuspid regurgitation, a diastolic murmur of pulmonary regurgitation and a right ventricular S3.
The standard diagnostic workup in developed countries includes a series of investigations to rule out the secondary causes. Additional tests are required to estimate the disease severity and plan the appropriate treatment. These include the cardiac catheterization, selective pulmonary angiography by direct injection of pulmonary arteries, high resolution CT scan, cardiac magnetic resonance, ABGs, nocturnal O2 saturation, etc. While most of the basic management is feasible in the Indian conditions, most of the newly introduced drugs are either not available or are available at costs that far exceed the paying capacity of an average citizen of a developing economy. An underdeveloped health insurance system adds further to the financial burden of the treatment.
Measures like formulation of guidelines for diagnosis and treatment of PAH, educating clinicians and scientists and making medications affordable to poor patients might ensue a breakthrough in the overall management of pulmonary hypertension.
Pulmonary Hypertension: Past Present and Future
Pulmonary Hypertension in India and the Developing World
Clinical and Genetic Aspects of Pulmonary Hypertension: An Overview
Evaluation of a Patient with Pulmonary Hypertension: What is Necessary for Diagnosis, Classification and Prognostication
Evidence-based Management of Pulmonary Arterial Hypertension
Assessment of Operability in Left to Right Shunts: Guidelines for the Practising Cardiologists
Pulmonary Hypertension Early After Congenital Heart Surgery: Prevention and Treatment
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- 31st October 2008
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Professor of Developmental Cardiology, Lead Clinician of the National Pulmonary Hypertension Service for Children, Great Ormond Street Hospital for Children, London WC1N 3 JH
"Dr. R Krishna Kumar is currently serving as the chief pediatric cardiologist at the Amrita Institute of Medical Sciences, Kochi, Kerala. He has also served as Consultant Cardiologist (Pediatric Cardiology) at Escorts Heart Institute & Research Centre, New Delhi. He has valuable contribution in over 80 national as well as international publications. Dr. Kumar has presented his papers at several conferences, both in India and abroad. He is among the pioneers in India in the field of CME. His major interest is in Pediatric Heart Disease in Developing Countries, Development of Inexpensive Devices and Strategies for catheter Interventions, and Education through Development of Teaching/Training Software for Cardiovascular Imaging."
Clinical Professor and Chief Pediatric Cardiologist, Amrita Institute of Medical Sciences, Kochi.