STUDY OF PULMONARY ARTERIAL HYPERTENSION IN RESPIRATORY DISORDERS *Hegde R.R., Bharambe R.S., Phadtare J.M. and Ramraje N.N. Department of Pulmonary Medicine, Grant Government Medical College, Mumbai-8 *Author for Correspondence ABSTRACT 1000 patients having respiratory complaints were selected randomly from OPD and were screened for various respiratory disorders (COPD, Asthma, ILD, Bronchiectasis, Post-infectious fibrosis & Occupational lung diseases) by symptomatology, history, clinical examination, Chest X ray & spirometry. (Standard criteria were used for diagnosis of various respiratory disorders). Echocardiography was performed in these patients for detection of pulmonary. The youngest was 26 yrs and the oldest was 78 years, mean age being 50 years.64% were male and 36% female. Out of 1000 patients, 50 i.e 5% had pulmonary.22 out of 400 COPD (5.5%),12 of 250 post infective fibrosis(4.8%), 9 of 50 ILD (18%), 4 of 90 bronchiectasis (4.4%) and 2 of 200 patients of bronchial asthma (1%) and 1 of 10 patients of pneumoconiosis(10%) had pulmonary. As the pulmonary arterial pressure increases, changes of the same are more pronounced on ECG and Chest X-ray. In chronic respiratory disorders, as FEV1 decreases, the severity of pulmonary arterial increases Key Words: Pulmonary Arterial Hypertension, Spirometry INTRODUCTION Pulmonary was first identified by Ernst von Romberg in 1891. Pulmonary Hypertension means an increase in the pulmonary arterial, venous and capillary pressure leading to shortness of breath, dizziness, fainting and other symptoms all of which are excacerbated by exertion.pulmonary can be a serious disease with markedly decreased exercise tolerance and heart failure.pulmonary arterial is defined as mean pulmonary arterial pressure (mpap) of more than 25 mm hg (Gibbs et al., 2001). Pulmonary can be due to diseases predominantly confined to the pulmonary vasculature as in pulmonary arterial. A pulmonary capillary occlusion pressure or a left ventricular end diastolic pressure of less than 15mm hg. increase in pulmonary vascular resistance of more than 3 wood units without significant respiratory or cardiac dysfunction. It can occur in association with disorders of the respiratory system or left heart.idiopathic pulmonary arterial is a rare disorder with an incidence of 2-3 /million/year (Rudrakanchana et al., 2001) other forms of Pulmonary are much commoner. In scleroderma the incidence is 6-60%, Rheumatoid arthritis it is 21%, SLE 4-14%, Portal 2-5%, Sicle cell disease 20-40%. 4% of people who have pulmonary embolism go on to develop chronic thromboembolic disease including pulmonary hyprtension. Pickwickian syndrome is commonly associated with pulmonary. MATERIALS AND METHODS 1000 patients having respiratory complaints were selected randomly from OPD and were screened for various respiratory disorders (COPD, Asthma, ILD, Bronchiectasis, Post-infectious fibrosis & Occupational lung diseases) by symptomatology, history, clinical examination, Chest X ray & spirometry. (Standard criteria were used for diagnosis of various respiratory disorders). All instructions were given to the participants regarding the study and the procedure. All the tests were carried out in the Pulmonary Function laboratory between 9am and 12 noons. The Pulmonary function tests were performed on computerized Pulmonary Function Test machine manufactured by Masterscreen Diffusion Jaeger. Patients who were smokers and had Obstructive Ventilatory defect i.e. forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) ratio less than 70% and FEV1 less than 80% with poor 230
bronchodilator reversibility were labelled as COPD Patients having a history of recurrent rhinitis, urticaria or a family history of asthma and having an obstructive ventilatory defect with good bronchodilator reversibility were labelled as Bronchial Asthma. In patients with Restrictive ventilatory defects with decreased DLCO, having honey combing or ground glassing on HRCT thorax, were labelled as Interstitial lung disease Bronchiectasis was diagnosed on the of history of chronic cough with sputum with exertional breathlessness confirmed by High Resolution Computed Tomography (HRCT) thorax. Silicosis was diagnosed by history of exposure to offending agent (i.e. silica) at place of work and confirmed by imaging (chest X ray and HRCT thorax). Patients with a past history of tuberculosis or childhood history of pneumonia having a restrictive ventilatory defect and showing fibrotic changes on High Resolution Computed Tomography (HRCT) thorax were diagnosed as post-infectious fibrosis. Screening was done by 2D ECHO to measure mpap i.e. if > 25 mm Hg, then labeled as Pulmonary. Pulmonary vascular resistance criteria were not applied as it is through invasive procedure (cardiac catheterization) which was refused by majority of patients. Chest X-ray PA view (showing right descending pulmonary of >20mm and right atrial enlargement), ECG changes ( p pulmonale and right axis deviation) were noted. All the above investigations were compared with severity of pulmonary. Inclusion Criteria 1. Adults above 18 years of age. 2. Either gender 3.2 D echo showing mpap of more than 25 mm hg Exclusion Criteria 1. Debilitated patient 2. Recent Myocardial Infarction, Congestive heart failure. 3. Valvular heart disease. RESULTS Distribution Age and Gender-wise Age Male Female Total 18-30 1 3 4 31-40 3 6 9 41-50 9 5 14 51-60 12 2 14 61-70 4 2 6 > 70 3-3 Total 32 18 50 12 10 8 6 4 Male Female 2 0 18-30 31-40 41-50 51-60 61-70 >70 231
Respiratory Disorder-wise COPD Asthma Post-infective fibrosis ILD Bronchiectasis Pneumoconiosis Incidence of Pulmonary Hypertension in Respiratory Disorders of the 1000 patients, 50 i.e 5% had pulmonary arterial. Patients with Pulmonary Patients without pulmonary Respiratory Disorder wise Incidence of Pulmonary Hypertension Respiratory Disorder No. of patients No.of patients with Pulmonary COPD 400 22 5.5 Post infective fibrosis 250 12 4.8 ILD 50 9 18 Bronchiectasis 90 4 4.4 Asthma 200 2 1 Pneumoconiosis 10 1 10 Total 1000 50 Percentage of patients with Pulmonary 232
Pulmonary Hypertension Severity-Wise Severity of Pulmonary Hypertension No. of patients Mild 37 Moderate 9 Severe 1 Very severe 3 Total 50 Mild Moderate Severe Very Severe Most of the patients had mild to moderate pulmonary Pulmonary Hypertension and ECG Changes Severity of PH No. of patients P pulmonale Right axis Both changes deviation Mild 37 27 10 9 Moderate 9 8 4 4 Severe 1 1 1 1 Very Severe 3 3 3 3 Total 50 39 18 17 78% of our patients had P pulmonale, 36% had right axis deviation and 34% had both the changes. It was observed that as the severity of Pulmonary Hypertension increases, the prevalence of the above ECG changes was 100% in severe and very severe group. Pulmonary Hypertension and Chest X Ray and 2D Echo Findings Severity of PH No.of patients Right descending Right atrial Both changes Pulmonary trunk enlargement > 20 mm Mild 37 29 24 22 Moderate 9 7 5 4 Severe 1 1 1 1 Very Severe 3 3 3 3 Total 50 40 33 30 233
80% of our patients had right descending pulmonary artery at its maximum diameter of more than 20 mm, 66% showed right atrial enlargementon 2 D echo and 60% showed both changes. As the severity of pulmonary increases, the prevalence of the above CXR findings was 100% in severe and very severe groups. Pulmonary Hypertension and Spirometry FEV1% >70 60-69 50-59 35-49 <35 Total No. patients of 2 3 5 13 27 50 Percentage 4 6 10 26 54 100 >70 60-69 50-59 35-49 <35 As the Pulmonary function deteriorates, the probability of having Pulmonary Hypertension increases. DISCUSSION 1000 patients with respiratory disorders were screened in our out patient department for pulmonary. Out of 1000 patients, 50 i.e 5% had pulmonary. 22 out of 400 COPD (5.5%),12 of 250 post infective fibrosis (4.8%), 9 of 50 ILD (18%), 4 of 90 bronchiectasuis(4.4%) and 2 of 200 patients of bronchial asthma (1%) and 1 of 10 patients of pneumoconiosis(10%) had pulmonary. The highest incidence of pulmonary was observed in ILD patients (18%). This was comparable to the study by Behr and Ryuz (2008). Overall incidence of Pulmonary Hypertension secondary to various respiratory disease was 5%.The incidence of pulmonary secondary to COPD was found to be 5.5%. This was lower as compared to study conducted by Naeije et al., (2003) who found it to be 10% (Naeije et al., 2003). It was inferred that as the severity of pulmonary increases the ECG changes in the form of P pulmonale and right axis deviation increased.only 23% of the patients with mild pulmonary had had both P pulmonale and 234
right axis deviation, but 100% of patients with severe and very severe disease had both changes.this was also true for CXR changes; only 60% of patients with mild pulmonary had simultaneously right descending pulmonary artery enlargement of more than 20 mm and right atrial enlargement but 100% of patients with severe and very severe disease had both the changes. Figure 1 Figure 2 Figure 3 235
As the pulmonary function parameters deteriorated, the number of patients having pulmonary increased i.e Amonst patients with FEV1 of more than 70% only 4% had pulmonary whereas 54% of patients with FEV1 less than 35% had pulmonary. Conclusion The occurrence of pulmonary arterial in patients having chronic respiratory disorders is approximately 5%. The highest occurrence was found in Interstitial lung disease i.e. 18% and the lowest i was found in Asthma i.e. 1%.Incidence in COPD was found to be 5.5%,4.8% in post infective fibrosis,4.4% in bronchiectasis and 10% in pneumoconiosis. As the severity pulmonary arterial Chest Xray, ECG and 2D echo changes are more pronounced increases, changes of the same are more pronounced on ECG and Chest X-ray. In chronic respiratory disorders, Pulmonary arterial and severity of respiratory disorders have a linear relationship REFERENCES Behr J and Ryuz J H (2008). Pulmonary Hypertension in ILD. European Respiratory Journal 31 1357-1367. Gibbs J R and Higenbottan J W et al., (2001). Recommendation on management of pulmonary in clinical practice British cardiac society guidelines and medical practice committee, and approved by British Thoracic society and British Society of Rheumatology. Heart 86(Suppl 1) i2-i13. Rudrakanchana N, Trembath RC and Morrell NW (2001). New insight into pathogenesis and treatment of primary pulmonary. Naejie R and Macknew et al., (2003). Pulmonary Circulation. In: Calverley P, Macknew, Pride P, Rennard S (editors) COPD 2 nd edition, London, Arnold, Health Sciences 228-242. Von Romberg and Ernst (1891-1892). Uber skierose Lungenorterie (in german). Deutch fur Archiv Klinische Medizin 48 197-206. 236