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Severe pulmonary artery hypertension due to an anomaly of the pulmonary arteries
Severe Pulmonary Artery Hypertension to an Anomaly
of the Pulmonary
Due
Arteries*
RACHEL SNITCOWSKY, M.D., ARMANDON. TOLEDO, M.D., WALDOMIROZANIOLO,M.D., H. X. DE BRITO, M.D., JORGEA. B. SEKEFF, M.D.,and ARTHURDE CARVALHOAZEVEDO,M.D.
AUGUSTO
Rio de Janeiro, Brazil
I
N ALL the classifications of the causes of pulmonary hypertension1-3 there is a group equivalent to what Goodwin calls “obliterative pulmonary hypertension,” comprising patients whose pulmonary vascular bed has become obliterated by disease. Several conditions capable of producing this obliteration are mentioned.r13-6 However, we are unable to find any reference to a case comparable to the one This fact and the opportunity of this article. of making not only a complete cardiologic study but also a study of the pulmonary function prompted us to publish it.
and 49.7 kg. in weight. There was no cyanosis or clubbing of the limbs, but there was slight pitting edema of the lower third of both legs. An abnormal presystolic venous wave was visible in the left supraclavicular fossa. The pulse was normal with a rate of 130/min. Blood pressure was lOOj65 mm. Hg. The apex beat was normal in character and location. There was a systolic lift at the second left interspace and Pz was palpable. Cardiac rhythm was regular. PZ was increased (4/4) and a small (2/4) musical mid-systolic murmur was heard along the left sternal border with a definite inspiratory accentuation. The respiratory rate was 32/min. Expansion, palpation and percussion of the thorax were normal. Vesicular breathing was normal. Scattered sibilant rales were heard in both lungs. Laboratory Examinations: Urine analysis showed Stool examination was normal. marked albuminuria. Blood count showed 5.5 million red cells per cu. mm. and hemoglobin of 1040/ Venous Pulse: The jugular pulse (Fig. 1) showed a giant a wave and an r wave replacing the systolic collapse of the pulse. Phonocardiogram: Small presystolic vibrations due to the auricular sound were recorded (Fig. 1). Roentgenogram and Fluoroscoby of the Thorax: There was normal mobility of the thoracic cage and of the diaphragm in the P-A view. In the profile view the right hemidiaphragm had a diminished excursion, and the expiratory collapse of the upper half of the right lung seemed incomplete. There was moderate cardiac enlargement with slight bulging of the upper part of the middle arch and marked accentuation of the lower right arch. The pulmonary arteries were visible and slightly dilated in both lung fields, more intensely so in the left hilum. The radiotranslucency was normal in both lung fields (Fig. 2). There was a regular sinus Electrocardiogram: rhythm with signs of right ventricular hypertrophy and severe right ventricular strain (Fig. 3).
CASE REPORT This 19 year old unmarried girl, first seen in March 1962, complained of severe fatigue and pain in her legs. Since childhood she had marked diminution of capacity for effort and had been unable to She was occafollow other children in their games. sionally seen by doctors who informed her parents that she had asthma. In April 1961 after a severe emotional trauma she Since then lost consciousness, fell and was cyanotic. dyspnea and fatigue were more severe, produced by the slightest effort and accompanied by palpitations She also and cyanosis of the lips and nail beds. Afterstarted feeling pain in her legs when walking. ward she had several episodes of dyspnea, palpitation During and loss of consciousness produced by effort. the last six months she also had constrictive precordial pain, lasting about 10 minutes, unrelated to effort. Vesperal edema of the lower limbs appeared in January 1962. Her family history was uneventful; there are 11 She was born of a normal siblings healthy and alive. pregnancy and during childhood had measles, whooping cough and chickenpox. Physical examination disclosed a normally built and apparently healthy young woman 161 cm. in height
* From the Department of Cardiology of the School of Post-Graduate Medicine of the Pontifical Catholic University of Rio de Janeiro and Department of Cardiology of the Institute for Retirement and Pensions of the Commercial This study was aided by a grant from the Employees (I.A.P.C.) Hospital N. S. das Vig&ias, Rio de Janeiro, Brazil. National Council for Research of Brazil. 542
THE
AMERICAN
JOURNAL
OF CARDIOLOGY
Anomaly
of Pulmonary
Arteries
543 TABLE
I
Catheterization
Data
--PreWXe--. Site
Syst.
Inferior vena CaYa Suoerior vena cava Rkht atrium
FIG. 1. Jugular venous pulse recorded simultaneor.sly with the phonocardioqam. showing giant a waves and an I wave during systole.
Right
ventricle
Right
pulmonary
artery
Left pulmonar)l
artery
Left pulmdnary Radial artery
“wedge”
25 21
16 16
19* 95 100 132* 110 109 135* 95 95
15* 44 46 51* 70 Z*
105
56 48 6 78
126%
96%
Oxygen consumption (cc./min.) Arteriovenous 01 difference (vol.‘%) Cardiac output (ml./min.) Cardiac index (L./min./M2.) Systolic output (ml.) Total pulmmary vascular resistance sec. cm-6.)
* Values t Values % Values
Roentgenogram
of the thorax.
FIG. 3. APRIL
1964
(mm. Hg) Dian. Meall
13 13 9% 7/22 t/21 16/25* ‘43 39 43* 41 40 62 67%
<-oxygenVol Sat. “:
s”o
6.4 6.4 5.8 4.8
36.9 36 9 33 5 27.7
6.2 6 5
35.8 37.5
6.1
35.2
6.7
13.9 14.3t 15.8%
I
38.7
80.7 82.6t 91.3%
Basal
After Digitalization
187.8 7.9 2,338.0 2.2 27.0
205.5 7.0 2,936.0 2.6 33.3
2,000.0
2,120.o
(dynes
20 min. after lanatoside C. with breathing of oxygen. 20 min. after lanatoside C and with breathing
oxygen.
Cardiac Catheterization: This procedure was done under slight sedation (Table I). In regard to the manipulation of the catheter, the only abnormality noted was that, while it was located in the horizontal part of the right pulmonary artery behind the cardiac shadow, it could not be directed into the right lung field; in several attempts there was always an impediment to its passage beyond the right hilum. The left pulmonary artery, however, was normally
Electrocardiogram.
544
Snitcowsky
et al.
Fig. 4. A, initial angiocardiogram showing blocking of contrast medium in the right hilum. B, close-up view of Figure A showing the blocking of contrast medium and diminution of caliber at the emergence of the artery for the upper lung (see arrow). C, repeat angiocardiogram showing great difference in size between the dilated main pulmonary artery and the right pulmonary artery. catheterized, and a normal “wedge” pressure was obtained. There was a severe hypertension in the right atrium, right ventricle and in both pulmonary arteries. Several slow pull-backs were done during
the recording of pressure in the right and left pulmonary arteries, and no gradient was found. The enddiastolic pressure was also markedly increased in the right ventricle, THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Anomaly ‘YABLE
of Pulmonar)-
I,
Studies of Pulmonary
Function
CXpWlCd VdlWS -
Observed V&YZS
SC of Expected
16 550 8,800 2,470
-22%
._-
Respiratory rate (rate/min) Tidal volume (cc.) Minute volume (cc./min.) Vital capacity (cc.)* Maximal cxpiratory flow 1st ser. (cc./%) 2nd WT. (cc./?‘,) 3rd sec. (cc./ %) Inspiratory reserve volume (cc.) Expiratory rexwe ~olumr (cc.) Functional residual aparity (cc.) Residual volume iVC.J Total lung rdpacity (cc.)t R. V./T.C. X 100 (%) Maximal breathing dpacity (l/min.)* Rate per minute Air velocity index Ambulatory ventilation (l./min.) Index Oxygen consumption (cc.,‘min./M”. in lOO’%t) Oxygen consumption Icc./min.M’.) Ventilatory equivalent (cc./a~r/loo cc. 02)
12-18 350-500 3,178 2,380 2,700 3,000
(
I
(75%) (85%) (95%)
1,580 1,900 2,030
1,370
1,859
1,720
1,138
1,170
4,288 26
3,640 33
95
48 40 0.7
*from
-16% -50%
140 132
120
2.0-3.2
-9%
4.0
Basal Recumbent
Theoretical values: treider; tfrom Cara.
-8%
22,300 0.46
0.30
r--Respiration
Oxygen capacity of blood (vol. %) Arterial oxygen (vol. %) Arterial saturation (%) Oxygen content whole blood (vol. %) Oxygen content of plasma (vol. %) COz pressure mtn. Hg (P&O*) Plasma pH Hematocrit (%) Hemoglobin (pm.)
(64%) (77%) (82%)
18.8 16.1 85.5
of Air----_ Effort 3 min. & 30 sec. 100%
18.8 16.4 87.0
32.2
29.7
01
18.8 17.1 91.0
and
Cournand;
31.6
t from
Kal-
Position of the patient was semirecumbent. All the values were corrected for body temperature, pressure and saturation (coefficient 1.075) with the exception of 01 consumption and rlimination of CO, which are standard (coefficient 0.899). Body surfarr: 1.52 M*.; body temperature: 36’ c.; room temperature: 25’ c. R. V./T.C. = residual volume/total capacity.
Oxygen analysis showed marked venous desaturation that, although improved, did not reach normal limits with respiration of oxygen. After the recording of the basal data an injection of 0.8 mg. of lanatoside C was made through the catheter and produced a marked increase in right ventricular and pulmonary artery pressures, a slight APRIL
I964
diminution of the A-\. oxygen dilferrncc anti ‘i sliglbt increase in the cardiac index and systolic: output (Table I). The response observed after digitalization \vas interpreted as indicative of right vc.ntricul;ur failure. iln:,iocar[lio,Sra~l: .l’he impossibility of cathctcrization of the right lung prompted us to inject contrast ‘I’his !\‘a~ done medium into the pulmonary artery. twice; the first injection, immediately upon completion of cardiac catheterization (Fig. 4), showed a marked diminution of the caliber of the horizontal p.+rt of the proximal right pulmonary artery. No branches were visible for the lower part 01 the right lung. The vascularization for the upper part of the right lung \vas visibly although of small caliber, but at its emcrgence from the right hilum, the upper lung artery seemed constricted. In the second angiocardiogram, made on another occasion, the dye was injected in the main pulmonary artery, which was markedly There was an adequate visualization of the dilated. left pulmonary artery branches, all of small caliber. At the emergence of the right pulmonary artery, clearly seen was the enormous difference between the small caliber of the horizontal part of the right pulmonary artery and the dilatation of the main pulmonary trunk. In the right lung field the pattern of visualization was similar to that of the first angiocal,diogram: Branches were visible only at the upper part. Pulmonary Function Study: The extreme incapacity of the patient precluded the study of the function of each lung. .4s can be seen from Table II the analysis showed a reduced maximal breathing capacity with a normal residual capacity. The abnormalities noted in the values of the maximal expiratory flow and of the maximal breathing capacity were interpreted as an indication of a difficulty in the air-flow, probably due to increased pulmonary resistance. COMMENTS
38.0 25.0 7.43 41.0 14.0 Baldwin
J -1.5
.kterien
The cardiopulmonary study of this young female patient demonstrated severe pulmonary hypertension and right ventricular failure. The full-blown picture of pulmonary hypertension with symptoms probably dating from childhood, the normal “wedge” pressure, absence of intracardiac shunts, and the results of the pulmonary function study seemed to indicate an obliterative type of pulmonary hypertension. The only clue to the underlying pathology was given by the angiocardiograms. The same degree of pulmonary hypertension was found in the main pulmonary artery, in the left branch and in the right branch, but in spite of this degree of hypertension the right pulmonary artery was diminutive in size. The pattern found at the emergence of the artery for the upper part of the right lung could fit that of socalled pulmonary artery stenosiP but not so
546
Snitcowsky
the size of the proximal right pulmonary artery : One would expect a marked dilatation of it, more so if we consider the pressure in it (109, on the contrary, its size was 391, whereas, markedly diminished. As was already mentioned, pressures were the same in the main and in the right pulmonary arteries. Thrombosis of the right pulmonary artery and some of its branches could also produce a similar angiocardiographic picture. Against thrombosis, however, was the history dating from childhood. In the cases of hypoplasia of pulmonary arteriesgsiO the plain x-ray films show an increased radiotranslucency in the affected lung, and frequently a shifting of the mediastinum to the affected site; but neither of these signs was found in this patient. In the cases of absence of the right pulmonary arteryg*iO there is also an increased radiotranslucency in the affected lung. In our patient, the radiotranslucency was normal, and although not opacified in the angiocardiograms, the pulmonary arteries were visible in the plain x-ray films. This case seemed to indicate that in patients with pulmonary hypertension of unknown a clinical diagnosis of “essential” etiology, hypertension should not be made until an angiocardiographic study is made and congenital or acquired anomalies of the pulmonary artery excluded. SUMM.~RY
The case of a 19 year old girl in heart failure with symptoms of severe pulmonary hypertension which dated from childhood is presented. The main findings in the cardiologic study were a normal radiotranslucency in both lungs, and severe right atria1 and ventricular hypertrophy in electrocardiogram. On cardiac catheterization there was a severe pulmonary hypertension with normal “wedge” pressure in the left lung. It was, however, impossible to advance the catheter into the distal branches of the right pulmonary artery : there was blocking of the catheter in the right hilum. The proximal right pulmonary artery pressure also was markedly elevated. Two angiocardiograms were made, and both showed a dilated main pulmonary artery, visible and constricted left pulmonary artery branches, and a diminutive
et al. right pulmonary artery branching from the dilated main artery, with visible branches only for the upper right lung. Pulmonary function study showed a reduced maximal breathing capacity due to the diminution of the vital capacity with a normal residual capacity. It is emphasized that, except data from the angiocardiograms and the catheterization of the right pulmonary artery, nothing in the clinical picture suggested the anomalies of the right pulmonary arteries. This fact would seem to indicate that every patient with a picture of pulmonary hypertension of unknown etiology should have angiocardiograms before a clinical diagnosis of “essential pulmonary hypertension” is made. ACKNOWLEDGMENT We gratefully acknowledge the data of the pulmonary function studies made by Dr. Henri Jouval in the Department of Pulmonary Diseases of the National Faculty of Medicine of the University of Brazil (Director: Prof. A. Ibiapina). REFERENCES 1. DALEY, R., GOODWIN, J. F. and STEINER, R. E. Clinical Disorders of the Pulmonary Circulation, p. 63. London, 1960. J. & A. Churchill, 2. SHEPHERD, J. T., EDWARDS,J. E., BURCHELL, H. B., SWAN, H. J. C. and WOOD, E. H. Clinical, physiological and pathological considerations in patients with idiopathic pulmonary hypertension. Brit. Heart J., 19: 70, 1957. 3. ADAMS, W. R. and VEITH, I. Pulmonary Circulation. Grune & Stratton, New York, 1959. 4. KNIDA., H.. , DAMMIN. , G.., HAYNES. F. W.. RAPPAPORT, E. and DEXTER, L. Primary pulmonary hypertension. Am. J. Med., 23: 166, 1957. 5. SLEEPER, J. C., ORGAIN, E. S. and MCINTOSH, H. D. Primary pulmonary hypertension. Circulation, 26 : 1358, 1962. 6. TOLEDO, A. N. et al. Estenose arterial pulmonar. Arch. Brasil. de Cardiologia (in press). 7. LUAN, L., D’SILVA, J. L., GASUL, B. M. and DILLON, R. F. Stenosis of the right main pulmonary artery. Clinical, angiocardiographic and catheterization findings in ten patients. Circulation, 21: 1116, 1960. 8. LSHR, H. VON, LOOOEN, F. and VIETEN, H. Die periphere Pulmonalstenose. Forts&r. Geb. R&tgenrtrahlen, 94: 285, 1961. 9. BELCHER, J. R., CAPEL, L., PATTINSON,J. N., and SMART, J. Hypoplasia of the pulmonary artery. Brit. J. Dis. Chest, 53: 253, 1959. 10. POOL, P. E., VOGEL, J. H. K. and BLOUNT,S. G., JR. Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am. J. Cardiol., 10: 706, 1962.
THE AMERICANJOURNAL OF CARDIOLOGY
of the Pulmonary
Due
Arteries*
RACHEL SNITCOWSKY, M.D., ARMANDON. TOLEDO, M.D., WALDOMIROZANIOLO,M.D., H. X. DE BRITO, M.D., JORGEA. B. SEKEFF, M.D.,and ARTHURDE CARVALHOAZEVEDO,M.D.
AUGUSTO
Rio de Janeiro, Brazil
I
N ALL the classifications of the causes of pulmonary hypertension1-3 there is a group equivalent to what Goodwin calls “obliterative pulmonary hypertension,” comprising patients whose pulmonary vascular bed has become obliterated by disease. Several conditions capable of producing this obliteration are mentioned.r13-6 However, we are unable to find any reference to a case comparable to the one This fact and the opportunity of this article. of making not only a complete cardiologic study but also a study of the pulmonary function prompted us to publish it.
and 49.7 kg. in weight. There was no cyanosis or clubbing of the limbs, but there was slight pitting edema of the lower third of both legs. An abnormal presystolic venous wave was visible in the left supraclavicular fossa. The pulse was normal with a rate of 130/min. Blood pressure was lOOj65 mm. Hg. The apex beat was normal in character and location. There was a systolic lift at the second left interspace and Pz was palpable. Cardiac rhythm was regular. PZ was increased (4/4) and a small (2/4) musical mid-systolic murmur was heard along the left sternal border with a definite inspiratory accentuation. The respiratory rate was 32/min. Expansion, palpation and percussion of the thorax were normal. Vesicular breathing was normal. Scattered sibilant rales were heard in both lungs. Laboratory Examinations: Urine analysis showed Stool examination was normal. marked albuminuria. Blood count showed 5.5 million red cells per cu. mm. and hemoglobin of 1040/ Venous Pulse: The jugular pulse (Fig. 1) showed a giant a wave and an r wave replacing the systolic collapse of the pulse. Phonocardiogram: Small presystolic vibrations due to the auricular sound were recorded (Fig. 1). Roentgenogram and Fluoroscoby of the Thorax: There was normal mobility of the thoracic cage and of the diaphragm in the P-A view. In the profile view the right hemidiaphragm had a diminished excursion, and the expiratory collapse of the upper half of the right lung seemed incomplete. There was moderate cardiac enlargement with slight bulging of the upper part of the middle arch and marked accentuation of the lower right arch. The pulmonary arteries were visible and slightly dilated in both lung fields, more intensely so in the left hilum. The radiotranslucency was normal in both lung fields (Fig. 2). There was a regular sinus Electrocardiogram: rhythm with signs of right ventricular hypertrophy and severe right ventricular strain (Fig. 3).
CASE REPORT This 19 year old unmarried girl, first seen in March 1962, complained of severe fatigue and pain in her legs. Since childhood she had marked diminution of capacity for effort and had been unable to She was occafollow other children in their games. sionally seen by doctors who informed her parents that she had asthma. In April 1961 after a severe emotional trauma she Since then lost consciousness, fell and was cyanotic. dyspnea and fatigue were more severe, produced by the slightest effort and accompanied by palpitations She also and cyanosis of the lips and nail beds. Afterstarted feeling pain in her legs when walking. ward she had several episodes of dyspnea, palpitation During and loss of consciousness produced by effort. the last six months she also had constrictive precordial pain, lasting about 10 minutes, unrelated to effort. Vesperal edema of the lower limbs appeared in January 1962. Her family history was uneventful; there are 11 She was born of a normal siblings healthy and alive. pregnancy and during childhood had measles, whooping cough and chickenpox. Physical examination disclosed a normally built and apparently healthy young woman 161 cm. in height
* From the Department of Cardiology of the School of Post-Graduate Medicine of the Pontifical Catholic University of Rio de Janeiro and Department of Cardiology of the Institute for Retirement and Pensions of the Commercial This study was aided by a grant from the Employees (I.A.P.C.) Hospital N. S. das Vig&ias, Rio de Janeiro, Brazil. National Council for Research of Brazil. 542
THE
AMERICAN
JOURNAL
OF CARDIOLOGY
Anomaly
of Pulmonary
Arteries
543 TABLE
I
Catheterization
Data
--PreWXe--. Site
Syst.
Inferior vena CaYa Suoerior vena cava Rkht atrium
FIG. 1. Jugular venous pulse recorded simultaneor.sly with the phonocardioqam. showing giant a waves and an I wave during systole.
Right
ventricle
Right
pulmonary
artery
Left pulmonar)l
artery
Left pulmdnary Radial artery
“wedge”
25 21
16 16
19* 95 100 132* 110 109 135* 95 95
15* 44 46 51* 70 Z*
105
56 48 6 78
126%
96%
Oxygen consumption (cc./min.) Arteriovenous 01 difference (vol.‘%) Cardiac output (ml./min.) Cardiac index (L./min./M2.) Systolic output (ml.) Total pulmmary vascular resistance sec. cm-6.)
* Values t Values % Values
Roentgenogram
of the thorax.
FIG. 3. APRIL
1964
(mm. Hg) Dian. Meall
13 13 9% 7/22 t/21 16/25* ‘43 39 43* 41 40 62 67%
<-oxygenVol Sat. “:
s”o
6.4 6.4 5.8 4.8
36.9 36 9 33 5 27.7
6.2 6 5
35.8 37.5
6.1
35.2
6.7
13.9 14.3t 15.8%
I
38.7
80.7 82.6t 91.3%
Basal
After Digitalization
187.8 7.9 2,338.0 2.2 27.0
205.5 7.0 2,936.0 2.6 33.3
2,000.0
2,120.o
(dynes
20 min. after lanatoside C. with breathing of oxygen. 20 min. after lanatoside C and with breathing
oxygen.
Cardiac Catheterization: This procedure was done under slight sedation (Table I). In regard to the manipulation of the catheter, the only abnormality noted was that, while it was located in the horizontal part of the right pulmonary artery behind the cardiac shadow, it could not be directed into the right lung field; in several attempts there was always an impediment to its passage beyond the right hilum. The left pulmonary artery, however, was normally
Electrocardiogram.
544
Snitcowsky
et al.
Fig. 4. A, initial angiocardiogram showing blocking of contrast medium in the right hilum. B, close-up view of Figure A showing the blocking of contrast medium and diminution of caliber at the emergence of the artery for the upper lung (see arrow). C, repeat angiocardiogram showing great difference in size between the dilated main pulmonary artery and the right pulmonary artery. catheterized, and a normal “wedge” pressure was obtained. There was a severe hypertension in the right atrium, right ventricle and in both pulmonary arteries. Several slow pull-backs were done during
the recording of pressure in the right and left pulmonary arteries, and no gradient was found. The enddiastolic pressure was also markedly increased in the right ventricle, THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Anomaly ‘YABLE
of Pulmonar)-
I,
Studies of Pulmonary
Function
CXpWlCd VdlWS -
Observed V&YZS
SC of Expected
16 550 8,800 2,470
-22%
._-
Respiratory rate (rate/min) Tidal volume (cc.) Minute volume (cc./min.) Vital capacity (cc.)* Maximal cxpiratory flow 1st ser. (cc./%) 2nd WT. (cc./?‘,) 3rd sec. (cc./ %) Inspiratory reserve volume (cc.) Expiratory rexwe ~olumr (cc.) Functional residual aparity (cc.) Residual volume iVC.J Total lung rdpacity (cc.)t R. V./T.C. X 100 (%) Maximal breathing dpacity (l/min.)* Rate per minute Air velocity index Ambulatory ventilation (l./min.) Index Oxygen consumption (cc.,‘min./M”. in lOO’%t) Oxygen consumption Icc./min.M’.) Ventilatory equivalent (cc./a~r/loo cc. 02)
12-18 350-500 3,178 2,380 2,700 3,000
(
I
(75%) (85%) (95%)
1,580 1,900 2,030
1,370
1,859
1,720
1,138
1,170
4,288 26
3,640 33
95
48 40 0.7
*from
-16% -50%
140 132
120
2.0-3.2
-9%
4.0
Basal Recumbent
Theoretical values: treider; tfrom Cara.
-8%
22,300 0.46
0.30
r--Respiration
Oxygen capacity of blood (vol. %) Arterial oxygen (vol. %) Arterial saturation (%) Oxygen content whole blood (vol. %) Oxygen content of plasma (vol. %) COz pressure mtn. Hg (P&O*) Plasma pH Hematocrit (%) Hemoglobin (pm.)
(64%) (77%) (82%)
18.8 16.1 85.5
of Air----_ Effort 3 min. & 30 sec. 100%
18.8 16.4 87.0
32.2
29.7
01
18.8 17.1 91.0
and
Cournand;
31.6
t from
Kal-
Position of the patient was semirecumbent. All the values were corrected for body temperature, pressure and saturation (coefficient 1.075) with the exception of 01 consumption and rlimination of CO, which are standard (coefficient 0.899). Body surfarr: 1.52 M*.; body temperature: 36’ c.; room temperature: 25’ c. R. V./T.C. = residual volume/total capacity.
Oxygen analysis showed marked venous desaturation that, although improved, did not reach normal limits with respiration of oxygen. After the recording of the basal data an injection of 0.8 mg. of lanatoside C was made through the catheter and produced a marked increase in right ventricular and pulmonary artery pressures, a slight APRIL
I964
diminution of the A-\. oxygen dilferrncc anti ‘i sliglbt increase in the cardiac index and systolic: output (Table I). The response observed after digitalization \vas interpreted as indicative of right vc.ntricul;ur failure. iln:,iocar[lio,Sra~l: .l’he impossibility of cathctcrization of the right lung prompted us to inject contrast ‘I’his !\‘a~ done medium into the pulmonary artery. twice; the first injection, immediately upon completion of cardiac catheterization (Fig. 4), showed a marked diminution of the caliber of the horizontal p.+rt of the proximal right pulmonary artery. No branches were visible for the lower part 01 the right lung. The vascularization for the upper part of the right lung \vas visibly although of small caliber, but at its emcrgence from the right hilum, the upper lung artery seemed constricted. In the second angiocardiogram, made on another occasion, the dye was injected in the main pulmonary artery, which was markedly There was an adequate visualization of the dilated. left pulmonary artery branches, all of small caliber. At the emergence of the right pulmonary artery, clearly seen was the enormous difference between the small caliber of the horizontal part of the right pulmonary artery and the dilatation of the main pulmonary trunk. In the right lung field the pattern of visualization was similar to that of the first angiocal,diogram: Branches were visible only at the upper part. Pulmonary Function Study: The extreme incapacity of the patient precluded the study of the function of each lung. .4s can be seen from Table II the analysis showed a reduced maximal breathing capacity with a normal residual capacity. The abnormalities noted in the values of the maximal expiratory flow and of the maximal breathing capacity were interpreted as an indication of a difficulty in the air-flow, probably due to increased pulmonary resistance. COMMENTS
38.0 25.0 7.43 41.0 14.0 Baldwin
J -1.5
.kterien
The cardiopulmonary study of this young female patient demonstrated severe pulmonary hypertension and right ventricular failure. The full-blown picture of pulmonary hypertension with symptoms probably dating from childhood, the normal “wedge” pressure, absence of intracardiac shunts, and the results of the pulmonary function study seemed to indicate an obliterative type of pulmonary hypertension. The only clue to the underlying pathology was given by the angiocardiograms. The same degree of pulmonary hypertension was found in the main pulmonary artery, in the left branch and in the right branch, but in spite of this degree of hypertension the right pulmonary artery was diminutive in size. The pattern found at the emergence of the artery for the upper part of the right lung could fit that of socalled pulmonary artery stenosiP but not so
546
Snitcowsky
the size of the proximal right pulmonary artery : One would expect a marked dilatation of it, more so if we consider the pressure in it (109, on the contrary, its size was 391, whereas, markedly diminished. As was already mentioned, pressures were the same in the main and in the right pulmonary arteries. Thrombosis of the right pulmonary artery and some of its branches could also produce a similar angiocardiographic picture. Against thrombosis, however, was the history dating from childhood. In the cases of hypoplasia of pulmonary arteriesgsiO the plain x-ray films show an increased radiotranslucency in the affected lung, and frequently a shifting of the mediastinum to the affected site; but neither of these signs was found in this patient. In the cases of absence of the right pulmonary arteryg*iO there is also an increased radiotranslucency in the affected lung. In our patient, the radiotranslucency was normal, and although not opacified in the angiocardiograms, the pulmonary arteries were visible in the plain x-ray films. This case seemed to indicate that in patients with pulmonary hypertension of unknown a clinical diagnosis of “essential” etiology, hypertension should not be made until an angiocardiographic study is made and congenital or acquired anomalies of the pulmonary artery excluded. SUMM.~RY
The case of a 19 year old girl in heart failure with symptoms of severe pulmonary hypertension which dated from childhood is presented. The main findings in the cardiologic study were a normal radiotranslucency in both lungs, and severe right atria1 and ventricular hypertrophy in electrocardiogram. On cardiac catheterization there was a severe pulmonary hypertension with normal “wedge” pressure in the left lung. It was, however, impossible to advance the catheter into the distal branches of the right pulmonary artery : there was blocking of the catheter in the right hilum. The proximal right pulmonary artery pressure also was markedly elevated. Two angiocardiograms were made, and both showed a dilated main pulmonary artery, visible and constricted left pulmonary artery branches, and a diminutive
et al. right pulmonary artery branching from the dilated main artery, with visible branches only for the upper right lung. Pulmonary function study showed a reduced maximal breathing capacity due to the diminution of the vital capacity with a normal residual capacity. It is emphasized that, except data from the angiocardiograms and the catheterization of the right pulmonary artery, nothing in the clinical picture suggested the anomalies of the right pulmonary arteries. This fact would seem to indicate that every patient with a picture of pulmonary hypertension of unknown etiology should have angiocardiograms before a clinical diagnosis of “essential pulmonary hypertension” is made. ACKNOWLEDGMENT We gratefully acknowledge the data of the pulmonary function studies made by Dr. Henri Jouval in the Department of Pulmonary Diseases of the National Faculty of Medicine of the University of Brazil (Director: Prof. A. Ibiapina). REFERENCES 1. DALEY, R., GOODWIN, J. F. and STEINER, R. E. Clinical Disorders of the Pulmonary Circulation, p. 63. London, 1960. J. & A. Churchill, 2. SHEPHERD, J. T., EDWARDS,J. E., BURCHELL, H. B., SWAN, H. J. C. and WOOD, E. H. Clinical, physiological and pathological considerations in patients with idiopathic pulmonary hypertension. Brit. Heart J., 19: 70, 1957. 3. ADAMS, W. R. and VEITH, I. Pulmonary Circulation. Grune & Stratton, New York, 1959. 4. KNIDA., H.. , DAMMIN. , G.., HAYNES. F. W.. RAPPAPORT, E. and DEXTER, L. Primary pulmonary hypertension. Am. J. Med., 23: 166, 1957. 5. SLEEPER, J. C., ORGAIN, E. S. and MCINTOSH, H. D. Primary pulmonary hypertension. Circulation, 26 : 1358, 1962. 6. TOLEDO, A. N. et al. Estenose arterial pulmonar. Arch. Brasil. de Cardiologia (in press). 7. LUAN, L., D’SILVA, J. L., GASUL, B. M. and DILLON, R. F. Stenosis of the right main pulmonary artery. Clinical, angiocardiographic and catheterization findings in ten patients. Circulation, 21: 1116, 1960. 8. LSHR, H. VON, LOOOEN, F. and VIETEN, H. Die periphere Pulmonalstenose. Forts&r. Geb. R&tgenrtrahlen, 94: 285, 1961. 9. BELCHER, J. R., CAPEL, L., PATTINSON,J. N., and SMART, J. Hypoplasia of the pulmonary artery. Brit. J. Dis. Chest, 53: 253, 1959. 10. POOL, P. E., VOGEL, J. H. K. and BLOUNT,S. G., JR. Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am. J. Cardiol., 10: 706, 1962.
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