Hospital Practice
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
Long-term Survival in Primary Pulmonary Hypertension Mohammad Z. Tahir To cite this article: Mohammad Z. Tahir (1992) Long-term Survival in Primary Pulmonary Hypertension, Hospital Practice, 27:11, 219-222, DOI: 10.1080/21548331.1992.11705530 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705530
Published online: 17 May 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [La Trobe University]
Date: 19 June 2016, At: 01:29
The Problem Patient
Long-term Survival in Primary Pulmonary Hypertension M 0 HAM MAD Z. TAHIR
Al-Hada Military Hospital, Ta'if, Saudi Arabia
Hospital Practice 1992.27:219-222.
Case Presentation A 50-year-old Saudi man, married and the father of 10 children, presented in 1981 with dyspnea on exertion (functional class III) ofthree months' duration, with left-sided atypical chest pain. There was no history of deep venous thrombosis, pulmonary emboli, ischemic or valvular heart disease, chronic obstructive pulmonary disease, drug abuse, or chronic liver disease. Family history was noncontributory. The patient appeared fit, was in no distress, and was not obese. His blood pressure was 120/70; pulse was 90 per minute andregularwithgood volume, as were all peripheral pulses. Jugular venous pressure was elevated at 10 em with a prominent A wave. A mild left parasternal impulse was noted on palpation of the precordium. On auscultation, a loud s4 was heard in the left parasternal region, indicating right atrial gallop with a grade 2/6 systolic ejection murmur in the pulmonary area with a loud pulmonary component of the second heart sound. Examination of the chest and abdomen did not reveal any abnormality. The ECG showed a heart rate of 90, normal sinus rhythm, right axis deviation, right ventricular hypertrophy, and right
atrial enlargement. Chest x-ray showed normal heart size with enlarged central pulmonary arteries and clear lung fields (Figure 1). Echocardiograms showed a dilated right ventricle (Figure 2) and right atrium with tricuspid regurgitation, pulmonary regurgitation, flat ejection fraction slope, and absent A wave of the pulmonic valve. Systolic flattening of the interventricular sep-
tum suggested right ventricular pressure overload (Figure 3). Left ventricle size was moderately reduced, with normal function. No wall motion abnormality was seen. A tentative clinical diagnosis of pulmonary hypertension was made, subject to further investigation. Laboratory investigations revealed the following values: hemoglobin 15.6, WBC 6,300 with normal differential, ery-
Figure 1. Hilar fullness and distal pruning of pulmonary vessels were the only abnormalities also evident in a patient who presented with dyspnea on exertion and left-sided chest pain. Heart size was normal and lung fields clear. Hospital Practice November 15, 1992
219
Hospital Practice 1992.27:219-222.
throcyte sedimentation rate 2. SMAC-20 was normal, RPR was nonreactive, and HBsAg was nonreactive. PTT and PT were normal.
220
Figure 2. A dilated right ventricle and posterior pericardia! effusion (PE) were recorded by M-mode echocardiography (parasternal long-axis view). The size of the left ventricular chamber, however, was normal.
On pulmonary function testing, FVC, FEV 1 , and FEV/FVC were normal; expiratory flow rate at 25% of FVC was 49% of predicted; and maximum midexpiratory flow at 25% to 75% of FVC was 43% of predicted. Peak expiratory flow rate decreased to 64% of predicted. The increase after bronchodilation was 20%, 78% of prediCted. The pulmonologist's diagnosis was small airways dysfunction, based on the decreased maximum expiratory and maximum midexpiratoryflow. Bilateral venography showed that both deep and superficial leg veins were filled, and no definite abnormalities were seen. Ultrasound examination of the ab-
Hospital Practice 1992.27:219-222.
domen was unremarkable, except for the incidental finding of a simple right renal cyst. Cardiac catheterization was done via the right femoral vein and artery in November 1981 without any complications and yielded the following values: right atrial pressure-arterial 13, venous 7, mean 6; right ventricular pressure-95/18; pulmonary artery pressure-95/22, mean 52; pulmonary wedge pressure-arterial 14, venous 14, mean 11; left ventricular pressure---118/10, aorta 118/74, mean95. There was no stenosis of any of the heart valves. No shunt was detected by oxygen sampling. Cardiac output was estimated at 6.3 L/min by the thermodilution technique. Cardiac index was 3.2 L/min/m2 • Pulmonary vascular resistance was elevated (6.5 mm
Figure 3. Systolic flattening of the interventricular septum (IVS), suggestive of pressure overload in the right ventricle, was notable with two~dimensional echocardiography (shown here in a short-axis view).
221
Hospital Practice 1992.27:219-222.
HgtL/min), and systemic vascular resistance was normal ( 14 mm Hg!L/min). Left ventriculogram biplane cineangiography at 30° and 60° by left anterior oblique projections revealed normal LV contractility and ejection fraction (0.55); no mitral regurgitation, ventricular septal defect, or patent ductus arteriosus was seen. A dilated right ventricle with poor contraction and an ejection fraction of 0.35 was seen on right ventriculography. The main pulmonary artery and the left and right branches were dilated, with pruning of the peripheral branches, representing the typical appearance of pulmonary hypertension. Primary pulmonary hypertension was diagnosed. Hydralazine treatment was monitored by pulmonary artery pressure with a Swan-Ganz catheter. No acute improvement in pulmonary hypertension was seen, and the patient was discharged on digoxin and diuretics. He did well on this treatment until 1991, when atrial fibrillation occurred. The ventricular rate remained under control, as the patient was still taking digoxin. With onset of atrial fibrilla-
tion, his systolic blood pressure dropped to 100 to 105. Currently, the patient is taking digoxin, furosemide, spironolactone, and warfarin. There is still slight decompensation of the right ventricle, but his symptoms are tolerable.
The Case in Context Primary pulmonary hypertension is uncommon, has a femaleto-male preponderance ( 1. 7: 1), and usually presents in the third to fourth decade. Its course is rapidly lethal, with mean survival of two to three years from onset of symptoms. A few patients have survived for more than 10 years. Functional class is inversely related to survival. Survival in patients with primary pulmonary hypertension and atrial fibrillation has not been reported, as lack of atrial contribution to left ventricular filling can result in sudden death. This is probably the first reported case of a patient who had primary pulmonary hypertension and onset of atrial fibrillation 10 years later without any hemodynamic collapse. Doppler studies have documented the marked contribution of ventricular filling by atrial systole. Loss
Selected Reading Hatano S, Strasser T (Eds): Primary Pulmonary Hypertension: Report on a WHO Meeting. World Health Organization, Geneva, 1975, pp 7-455 Dresdale DT, Schultz M, Michtom RJ: Primary pulmonary hypertension: I. Clinical and hemodynamic study. Am J Med 11: 686, 1951 Rich Setal: Primary pulmonary hypertension: A national prospective study.AnnlnternMed 107:216. 1987 Fuster Vet al: Primary pulmonary hypertension: Natural history and the importance of thrombosis. Circulation 70:580, 1984 Reitz BA: Heart-lung transplantation. In Pulmonary Diseases and Disorders, 2nd ed, Fishman AP (Ed). McGraw-H111, New York, 1987, pp 2459-2466 Rich S: Primary pulmonary hypertension. Prog Cardiovasc Dis 31: 205, 1988
222
Hospital Practice November 15, 1992
of atrial kick can result in hemodynamic collapse and sudden cardiac death. Our patient presented with dyspnea on exertion (functional class III) with a right ventricular ejection fraction of 0.35. Survival at this stage averages 3.5 years, but our patient is still alive after approximately 11 years. The usual clinical course of primary pulmonary hypertension is one of rapid deterioration; by the time the diagnosis is made, the clinical and hemodynamic changes of severe pulmonary hypertension are irreversible. Most patients (75%) present with dyspnea on exertion. Other clinical features, such as dizziness, syncope, chest pain, and ankle swelling, are infrequent. For the most part, deaths are due to right ventricular failure; death from pneumonia and sudden death are infrequent. Functional class at the time of presentation is a strong predictor of survival. In some studies, pulmonary arterial oxygen saturation of 63% or less was a poor prognostic factor. Treatment of primary pulmonary hypertension has thus far been disappointing. Vasodilators have a favorable acute effect but are not useful as a maintenance regimen. Our patient did not show any beneficial effect even with acute treatment. Diuretics give symptomatic relief of right-sided congestion. A significant beneficial effect of anticoagulant therapy on survival has been reported. Heart-lung transplantation has shown promising results, but should be reserved for patients with advanced disease and an expected survival of less than one year. D
Dr. Tahir is Consultant Cardiologist, AI-Hada Military Hospital, Ta'if, Saudi Arabia.
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
Long-term Survival in Primary Pulmonary Hypertension Mohammad Z. Tahir To cite this article: Mohammad Z. Tahir (1992) Long-term Survival in Primary Pulmonary Hypertension, Hospital Practice, 27:11, 219-222, DOI: 10.1080/21548331.1992.11705530 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705530
Published online: 17 May 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [La Trobe University]
Date: 19 June 2016, At: 01:29
The Problem Patient
Long-term Survival in Primary Pulmonary Hypertension M 0 HAM MAD Z. TAHIR
Al-Hada Military Hospital, Ta'if, Saudi Arabia
Hospital Practice 1992.27:219-222.
Case Presentation A 50-year-old Saudi man, married and the father of 10 children, presented in 1981 with dyspnea on exertion (functional class III) ofthree months' duration, with left-sided atypical chest pain. There was no history of deep venous thrombosis, pulmonary emboli, ischemic or valvular heart disease, chronic obstructive pulmonary disease, drug abuse, or chronic liver disease. Family history was noncontributory. The patient appeared fit, was in no distress, and was not obese. His blood pressure was 120/70; pulse was 90 per minute andregularwithgood volume, as were all peripheral pulses. Jugular venous pressure was elevated at 10 em with a prominent A wave. A mild left parasternal impulse was noted on palpation of the precordium. On auscultation, a loud s4 was heard in the left parasternal region, indicating right atrial gallop with a grade 2/6 systolic ejection murmur in the pulmonary area with a loud pulmonary component of the second heart sound. Examination of the chest and abdomen did not reveal any abnormality. The ECG showed a heart rate of 90, normal sinus rhythm, right axis deviation, right ventricular hypertrophy, and right
atrial enlargement. Chest x-ray showed normal heart size with enlarged central pulmonary arteries and clear lung fields (Figure 1). Echocardiograms showed a dilated right ventricle (Figure 2) and right atrium with tricuspid regurgitation, pulmonary regurgitation, flat ejection fraction slope, and absent A wave of the pulmonic valve. Systolic flattening of the interventricular sep-
tum suggested right ventricular pressure overload (Figure 3). Left ventricle size was moderately reduced, with normal function. No wall motion abnormality was seen. A tentative clinical diagnosis of pulmonary hypertension was made, subject to further investigation. Laboratory investigations revealed the following values: hemoglobin 15.6, WBC 6,300 with normal differential, ery-
Figure 1. Hilar fullness and distal pruning of pulmonary vessels were the only abnormalities also evident in a patient who presented with dyspnea on exertion and left-sided chest pain. Heart size was normal and lung fields clear. Hospital Practice November 15, 1992
219
Hospital Practice 1992.27:219-222.
throcyte sedimentation rate 2. SMAC-20 was normal, RPR was nonreactive, and HBsAg was nonreactive. PTT and PT were normal.
220
Figure 2. A dilated right ventricle and posterior pericardia! effusion (PE) were recorded by M-mode echocardiography (parasternal long-axis view). The size of the left ventricular chamber, however, was normal.
On pulmonary function testing, FVC, FEV 1 , and FEV/FVC were normal; expiratory flow rate at 25% of FVC was 49% of predicted; and maximum midexpiratory flow at 25% to 75% of FVC was 43% of predicted. Peak expiratory flow rate decreased to 64% of predicted. The increase after bronchodilation was 20%, 78% of prediCted. The pulmonologist's diagnosis was small airways dysfunction, based on the decreased maximum expiratory and maximum midexpiratoryflow. Bilateral venography showed that both deep and superficial leg veins were filled, and no definite abnormalities were seen. Ultrasound examination of the ab-
Hospital Practice 1992.27:219-222.
domen was unremarkable, except for the incidental finding of a simple right renal cyst. Cardiac catheterization was done via the right femoral vein and artery in November 1981 without any complications and yielded the following values: right atrial pressure-arterial 13, venous 7, mean 6; right ventricular pressure-95/18; pulmonary artery pressure-95/22, mean 52; pulmonary wedge pressure-arterial 14, venous 14, mean 11; left ventricular pressure---118/10, aorta 118/74, mean95. There was no stenosis of any of the heart valves. No shunt was detected by oxygen sampling. Cardiac output was estimated at 6.3 L/min by the thermodilution technique. Cardiac index was 3.2 L/min/m2 • Pulmonary vascular resistance was elevated (6.5 mm
Figure 3. Systolic flattening of the interventricular septum (IVS), suggestive of pressure overload in the right ventricle, was notable with two~dimensional echocardiography (shown here in a short-axis view).
221
Hospital Practice 1992.27:219-222.
HgtL/min), and systemic vascular resistance was normal ( 14 mm Hg!L/min). Left ventriculogram biplane cineangiography at 30° and 60° by left anterior oblique projections revealed normal LV contractility and ejection fraction (0.55); no mitral regurgitation, ventricular septal defect, or patent ductus arteriosus was seen. A dilated right ventricle with poor contraction and an ejection fraction of 0.35 was seen on right ventriculography. The main pulmonary artery and the left and right branches were dilated, with pruning of the peripheral branches, representing the typical appearance of pulmonary hypertension. Primary pulmonary hypertension was diagnosed. Hydralazine treatment was monitored by pulmonary artery pressure with a Swan-Ganz catheter. No acute improvement in pulmonary hypertension was seen, and the patient was discharged on digoxin and diuretics. He did well on this treatment until 1991, when atrial fibrillation occurred. The ventricular rate remained under control, as the patient was still taking digoxin. With onset of atrial fibrilla-
tion, his systolic blood pressure dropped to 100 to 105. Currently, the patient is taking digoxin, furosemide, spironolactone, and warfarin. There is still slight decompensation of the right ventricle, but his symptoms are tolerable.
The Case in Context Primary pulmonary hypertension is uncommon, has a femaleto-male preponderance ( 1. 7: 1), and usually presents in the third to fourth decade. Its course is rapidly lethal, with mean survival of two to three years from onset of symptoms. A few patients have survived for more than 10 years. Functional class is inversely related to survival. Survival in patients with primary pulmonary hypertension and atrial fibrillation has not been reported, as lack of atrial contribution to left ventricular filling can result in sudden death. This is probably the first reported case of a patient who had primary pulmonary hypertension and onset of atrial fibrillation 10 years later without any hemodynamic collapse. Doppler studies have documented the marked contribution of ventricular filling by atrial systole. Loss
Selected Reading Hatano S, Strasser T (Eds): Primary Pulmonary Hypertension: Report on a WHO Meeting. World Health Organization, Geneva, 1975, pp 7-455 Dresdale DT, Schultz M, Michtom RJ: Primary pulmonary hypertension: I. Clinical and hemodynamic study. Am J Med 11: 686, 1951 Rich Setal: Primary pulmonary hypertension: A national prospective study.AnnlnternMed 107:216. 1987 Fuster Vet al: Primary pulmonary hypertension: Natural history and the importance of thrombosis. Circulation 70:580, 1984 Reitz BA: Heart-lung transplantation. In Pulmonary Diseases and Disorders, 2nd ed, Fishman AP (Ed). McGraw-H111, New York, 1987, pp 2459-2466 Rich S: Primary pulmonary hypertension. Prog Cardiovasc Dis 31: 205, 1988
222
Hospital Practice November 15, 1992
of atrial kick can result in hemodynamic collapse and sudden cardiac death. Our patient presented with dyspnea on exertion (functional class III) with a right ventricular ejection fraction of 0.35. Survival at this stage averages 3.5 years, but our patient is still alive after approximately 11 years. The usual clinical course of primary pulmonary hypertension is one of rapid deterioration; by the time the diagnosis is made, the clinical and hemodynamic changes of severe pulmonary hypertension are irreversible. Most patients (75%) present with dyspnea on exertion. Other clinical features, such as dizziness, syncope, chest pain, and ankle swelling, are infrequent. For the most part, deaths are due to right ventricular failure; death from pneumonia and sudden death are infrequent. Functional class at the time of presentation is a strong predictor of survival. In some studies, pulmonary arterial oxygen saturation of 63% or less was a poor prognostic factor. Treatment of primary pulmonary hypertension has thus far been disappointing. Vasodilators have a favorable acute effect but are not useful as a maintenance regimen. Our patient did not show any beneficial effect even with acute treatment. Diuretics give symptomatic relief of right-sided congestion. A significant beneficial effect of anticoagulant therapy on survival has been reported. Heart-lung transplantation has shown promising results, but should be reserved for patients with advanced disease and an expected survival of less than one year. D
Dr. Tahir is Consultant Cardiologist, AI-Hada Military Hospital, Ta'if, Saudi Arabia.
