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Pregnancy with tetralogy and pentalogy of Fallot
Case Reports Pregnancy with Tetralogy
and
Pentalogy of Fallot* COMMANDERWILLIAM J. JACOBY, JR., MC, usNt Portsmouth,
LTHOUGH persons with the abnormalities of Fallot probably constitute the largest group of patients with cyanotic congenital heart disease surviving to adulthood, little has appeared in the literature concerning pregnancy in this condition. The scant clinical experience in such patients was summarized by Mendelson’ who reviewed the 33 patients reported prior to 1959. Since that time reports of 8 patients have been added to the literature.24 The majority constitutesun operated cases; however, about 25 per cent have had palliative surgery including pulmonary valvulotomy or a shunt procedure. The lack of complete clinical and obstetric information prevents determination of the exact maternal and fetal mortality; however, Mendelson recorded 3 maternal and 11 fetal deaths with approximately one-third of the fetal losses resulting from interruption of pregnancies. The 5 cases in this paper, which represent form one of the largest seven pregnancies, single series reported to date. Based on the experience gained in caring for these patients the management and the significant factors affecting maternal and fetal prognosis will be discussed.
Virginia
and diminished cyanosis. In 1954 (age 16) a right frontal lobe abscess developed which was treated successfully by surgical drainage and antibiotics with little neurologic residual. From 1954 to 1959 she felt remarkably well. In March 1959 the patient was examined in the Maternity Cardiac Clinic, and the diagnosis of intrauterine pregnancy was estabAt this time her hematocrit was 46 per cent. lished. In April she was admitted to the hospital at 28 weeks of pregnancy during the peak blood volume period. Therapy included restricted activity and digitalization. In July (37 weeks) fluid retention developed but responded to restricted sodium diet and chlorothiazide. On July 13, (38 weeks) the patient went into labor. Nasal oxygen was administered and leg wraps applied. Fetal heart tones were recorded during labor; however, just prior to delivery none were audible, and she delivered a stillborn infant. In the early postpartum period, tachycardia, a ventricular gallop, bilateral basal rales and peripheral edema developed. She responded slowly to complete bedrest, digitalis, chlorothiazide and oxygen therapy. During the next two weeks gradual ambulation was permitted. She was discharged to the outpatient department one month postpartum. Physical Examination: The patient was thin and cyanotic. Blood pressure was 160/40-O mm. Hg. There were marked cervical pulsations bilaterally but no hepatojugular reflux. A healed left thoracotomy scar was present. The maximal palpable impulse was 12 cm. from the midsternal line in the fifth intercostal space. The rhythm was normal sinus. The first sound was increased and loudest at the apex. The second sound was of slightly increased intensity A third sound in diastole was noted at and unsplit. the apex along with an ejection sound at the left
A
CASE REPORTS CASE 1. A 21 year old white woman was noted to Dyspnea, squatbe cyanotic since shortly after birth. ting and retarded growth were present during childIn 1948 (age 10) a Blalock-Taussig shunr was hood. performed with marked symptomatic improvement
* From the Cardiology Branch (Department of Medicine) and Cardiopulmonary Laboratory, U. S. Naval Hospital, Portsmouth, Va. The opinions or assertions contained herein are those of the author and are not to be construed as official or reflecting the views of the Navy Department or of the Naval Service at large. i Present address: U. S. Naval Hospital, Great Lakes, Ill. 866
THE
AMERICAN JOURNALOF
CARDIOLOGY
Pregnancy Case 1
Case 2
and Tetralogy Case 3
of Fallot Case 4
867 case 5
aVR
aVL
aVF
FIG. 1. Electrocardiograms in the 5 cases. Note the direction of the QRS loop in the frontal plane and the variations in the mean QRS and ST-T wave vectors. VOLUME
14,
DECEMBER
1964
4
stt’rnal bordvr. .\ Sradc 3 (I h:~r\h ‘J *tolic LIIII~I~LU Mas heard along ~hc Icft ~tc‘rnal bordu. loudest in A diastolic the third and fortrth intercostal spaccq. ;2 rtunble follo\ted the third sound a~ the apex. grade 4 continuous murmur was audible in the second and third left intercostal spaces and transPeriphmitted to the intrascapular area posteriorly. eral pulses were full and the water-hammer type. The liver was palpable at the costal margin. No splenomegaly nor peripheral edema was present. The electrocardiogram (Fig. 1) demonstrated a first degree A-V block, right atria1 hypertrophy and right ventricular hypertrophy and strain. The roentgenogram of the chest (Fig. 2) demonstrated combined ventricular atria1 enlargement, right hypertrophy with the major ventricular involvement being most likely right-sided, increased peripheral pulmonav vasculature with the left pulmonary artery more prominent than the right, and a normal aortic configuration. The barium-filled esophagus was deviated posteriorly by the left atria1 enlargement. The cardiac catheterization data are presented in
‘l’abk I. .I‘he patient time of study.
5 was in mild heart failure at the
CME 2. l’his was a 39 year old white woman born of a normal pregnancy and delivery. A heart murmur and cyanosis were present at birth. Her growth and development were slightly retarded; however, she made adequate progress, suffering only easy fatigue, exertional dyspnea and increasing cyanosis with strenuous activity. At age 36 (1956) she had a full term, normal pregnancy with no complications. During the next two years the patient experienced some increasing dyspnea on exertion; however, she was able to perform her household duties. Cardiac catheterization was performed in 1958, and repeated in 1959. The patient’s second pregnancy occurred in 1959. Her management included bimonthly visits to the outpatient clinic until the last trimester when she was examined weekly. A restricted sodium diet was rigidly followed, and she required no oral diuretics. Her hematocrit was 58 per cent. She was admitted to the hospital in February 1960, two weeks prior to delivery, and her activity
Pregnancy
and Tetralogy
of Fallot
869
TABLE I Cardiac
Case
RA (mean)
Pressures--(mm. Hg) RV
PA
BA 130/llO/-
1 2
7 4
120/17 110/o
3u;;o
3
4
88/5
1415
4 5
4 4
130/O 92/P
14/5
.
Catheterization
Data
Dye Dilution Curve * (Shunt)
Arterial Saturation (a/o)
82/50
87
,..
77 a7
143190
L-R L-R R-L L-R R-L
86 90
L:R R-L
Angiocardiogram VSD PS (infundibular), PS (valvular and infundibular), VSD
PS, VSD
PS = pulmonic stenosis; VSD = ventricular septal defect; RA = right atrium; RV = right ventricle; PA = pulmonary artery; BA = brachial artery. * Dve dilution curves were uerformed with iniection into the RA except in Case 3 where injection was into the RV. Sampling was from the brachi’al artery. was markedly restricted. At the onset of labor, nasal oxygen was administered, and leg wraps were applied. The delivery was performed with meperidine analgesia, pudendal block and the application of low forceps. The infant was a full term, normal male, weighing 4 lbs. 8 oz. In the postpartum period she received prophylactic antiAmbulation was gradually increased during biotics. her two week hospitalization. Subsequent to her second pregnancy, the patient’s cardiovascular status had remained essentially unchanged. Physical Examination: The patient was well developed and slightly obese and demonstrated cyanosis of the lips, fingers and toes. Blood pressure was 130/90 mm. Hg. There was no cervical venous The lungs were clear. A systolic thrill distention. was palpable along the left upper sternal border and Normal sinus a right ventricular lift was present. The first sound was normal and rhythm was noted. loudest at the apex. A second sound was widely split with diminution of the pulmonary component. A grade 4 harsh systolic murmur was present over the entire precordium, loudest along the left sternal border in the second, third and fourth intercostal PeriphNo diastolic murmurs were audible. spaces. eral pulses were normal and equal bilaterally. There was no hepatosplenomegaly nor peripheral Mild clubbing of the fingers was noted. edema. The electrocardiogram (Fig. 1) showed right ventricular hypertrophy. The roentgenogram of the chest (Fig. 2) demonstrated a normal transverse cardiac diameter with prominent left pulmonary artery and diminished peripheral pulmonary vasculature. The cardiac catheterization findings were as noted in through an Table I. The catheter was manipulated atria1 septal defect from the right atrium to the left atrium and subsequently from the right ventricle to the aorta. VOLUME
14, DECEMBER
1964
CASE 3. This was a 20 year old Negro woman who gave a vague history; however, as a child she was At age 17 a murmur was noted treated for asthma. but not evaluated further until her first pregnancy in The initial pregnancy was uneventful from a 1960. cardiovascular standpoint; she delivered a full term normal infant. Her cardiac status remained unchanged, and cardiac catheterization was performed She had a second uneventful full term in July 1961. pregnancy in 1962. Instructions for routine outpatient visits and a restricted sodium intake were followed. No diuretics were required. Her hematocrit was 45 per cent. She had a spontaneous normal delivery with the infant weighing 5 lb. 6 oz. Physical Examination: The patient had mild cyanosis and clubbing of the fingers and toes. Blood pressure was 116/74 mm. Hg. No cervical venous distention was present. The lungs were clear. A systolic thrill was palpable in the third intercostal space along the left sternal border. The second sound was split with a diminished second component. A grade 3 systolic murmur was present over the precordium loudest along the left sternal border in the third intercostal space. No diastolic murmurs were Peripheral pulses were normal and equal. audible. There was no hepatosplenomegaly nor peripheral edema. The electrocardiogram (Fig. 1) demonstrated right ventricular hypertrophy. The roentgenogram of the chest (Fig. 2) illustrated right ventricular enlargement, slightly diminished pulmonary vasculature and the aorta descending on the right. The cardiac catheterization data are shown in Table I. A cineangiocardiogram was not obtained due to poor cooperation of the patient requiring termination of the study. CASE 4.
This was a 27 year old white woman born
Jacoby of a normal pregnancy and delivery. Shortly after her birth the family was informed that she had cyanotic congenital heart disease. During childhood frequent respiratory infections and squatting were denied but with increased activity she was easily fatigued and experienced dyspnea. Her cyanosis and clubbing remained unchanged. In 1956 a Her subsecardiac catheterization was performed. quent course had been uneventful except for the continued fatigue and dyspnea on exertion. On two occasions she had sharp anterior chest pain which was relieved by rest. This pain did not radiate to the arms or abdomen and was not associated with dyspnea, sweating or palpitations. She became pregnant During her twentieth week of pregnancy in 1961. she noted peripheral edema and was admitted to the hospital for further evaluation. Therapy included bedrest, restricted sodium diet and chlorothiazide. An initial response of weight loss ensued and then an An original uneventful hospitalization of five weeks. hematocrit was 60 per cent but was later maintained between 50 and 52 per cent. She was discharged to return in four days for rehospitalization. On the day of admission she experienced a sense of fullness in the throat followed by minimal hemoptysis. Coagulation studies were normal and the chest x-ray films remained unchanged from the initial films. During the next 48 hours minimal hemoptysis occurred. On the third hospital day massive pulmonary hemorrhage resulted in death. Physical Examination: The patient was moderately well developed and nourished and displayed diffuse cyanosis with no variation between the upper and lower extremities and equal clubbing of both fingers and toes. Blood pressure was 112/58 mm. Hg. The fundi revealed vascular engorgement and marked prominence of choroidal vessels. Auriculoventricular pulsations were present in the neck. There was slight prominence of the upper sternal region with a thrill palpable in the left second and third intercostal spaces. The lungs were clear. Normal sinus rhythm was present. The first sound was normal and loudest at the apex; the second, of slightly increased intensity, loudest at the third left intercostal space and with no evidence of splitting. An ejection sound was present along the left sternal border. A grade 3/6 harsh, systolic murmur was audible over the entire precordium but loudest in the second and third left intercostal spaces. No diastolic murmurs were audible. Peripheral pulses were normal and equal bilaterally. There was no hepatosplenomegaly nor peripheral edema. The electrocardiogram (Fig. 1) revealed right ventricular hypertrophy and strain. The roentgenogram of the chest (Fig. 2) showed dis-
first
sound
The
second
placement
unsplit,
of the heart
encroachment of right artery
in
the
ventricular was not well
pulmonary
vasculature
toward
the left.
retrosternal
space
enlargement. visualized,
The and
was decreased.
the
There
was
suggestive pulmonary peripheral
The cardiac catheterization data are illustrated in Table I. Autopq: ‘I’he heart demonstrated pulmonary valvular (bicuspid valve) and infundibular stenosis, a ventricular septal defect, right ventricular hypertrophy and an atria1 septal defect. Pulmonary atelectasis and hemorrhage with a clot-cast of the bronchial tree was present. However, no specific area of hemorrhage or infarction was noted. There were no abnormal fetal cardiac defects. CASE 5. This was a 19 year old, white woman who had been aware of heart disease during her entire life and stated that she was a “blue baby.” During her early childhood her activity was restricted, and after exertion she was observed to squat. Her growth and development had been retarded, and she had suffered from multiple skeletal deformities. She had noted dyspnea on exertion and occasional increased cyanosis. In January 1961, she was admitted for cardiac catheterization. The patient was re-evaluated in December 1961, at which time she was 16 weeks pregnant. She tolerated her pregnancy well following a restricted sodium diet, and no diuretics were required. Her hematocrit was 50 per cent. During hospitalization from the twentysixth to the thirty-fourth her activity was severely restricted, and she was readmitted at 37 weeks of pregnancy. At the onset of labor, nasal oxygen was administered and leg wraps applied. An uncomplicated delivery was performed with meperidine analgesia, pudendal block and the application of low forceps. The infant weighed 4 lb. 3 oz. In the postpartum period several episodes of tachycardia, weakness and increased cyanosis with no loss of consciousness occurred. During these episodes sedation and an oxygen tent were employed with no other specific therapy. The attacks subsided, and ambulation was gradual. The patient was discharged in her third postpartum week. Physical Examination: The patient was short and moderately well nourished with obvious skeletal Blood pressure was 112/80 mm. Hg. deformities. The fundi were normal. There was no cervical A systolic thrill was palpable in venous distention. the suprasternal notch. The chest was deformed to the right by a thoracic scoliosis and associated lordosis which resulted in prominence of the right scapula and elevation of the right shoulder. The lungs were clear. A right ventricular lift and thrill were palpable along the right sternal border and midsternal
area
spaces.
A
and
in the second sinus was
to the fourth
arrhythmia normal
sound there
was
and
was
precordium,
loudest to the fourth
along
sternal
THE
border.
AMERICAN
the
intensity
with
spaces
No diastolic
JOURNAL
and
over the
midsternal
intercostal
The apex.
respiration.
was audible
in the
from the second the right
at
of increased
A grade 4 harsh systolic murmur entire
loudest
was no change
intercostal
present.
area and mur-
OF CARDIOLOGY
Pregnancy and Tetralogy of Fallot murs or gallops were audible. Peripheral pulses were normal and equal bilaterally. There was no There was cyanosis and clubhepatosplenomegaly. bing of the digits of the upper and lower extremities. A band constriction of the middle segment of the fourth left finger was present. The electrocardiogram (Fig. 1) demonstrated right ventricular hypertrophy. The roentgenogram of the chest (Fig. 2) revealed a thoracic scoliosis with a shift of the cardiac shadow to the right and right atria1 and right ventricular enlargement. The main pulmonary artery segment was not well visualized; however, there was slight prominence of the left pulmonary artery. The peripheral pulmonary vasculature was markedly reduced. The aorta was of normal configuration. The cardiac catheterization data are shown in Table I. An angiocardiogram was not performed. DISCUSSION Five cases of pregnancy in women with combined pulmonic stenosis, dextroposition of the aorta and a ventricular septal defect are presented. These lesions comprise the hemodynamitally significant malformations of a tetralogy of In two instances there was an associated Fallot. atria1 septal defect resulting in a pentalogy. Survival of these patients to the childbearing age is no longer unusual as evidenced by the increasing number of cases observed on large obstetrical services. In past decades cyanotic congenital heart disease was an indication for interruption of the pregnancy; however, this is not a valid concept since many of these patients will tolerate pregnancy under proper supervision. MANAGEMENT The medical management during childbearing must be stringent and uncompromising. Strict attention must be directed during frequent periodic outpatient visits to diet, weight gain, peripheral edema, anemia, cyanosis, syncope and changes in the physical examination or the electrocardiogram. Hospitalization during the period of peak blood volume and for one to two weeks prior to delivery may be necessary. Oral diuretics in conjunction with salt restriction are most useful in controlling At the time of delivery, leg fluid retention. prophylactic antibiotics and wraps, oxygen, Analavoidance of hypotension are indicated. gesia and pudendal block anesthesia at delivery were employed in the present series of patients. During the postpartum period the same careful attention to hypotension, anoxia, anemia and VOLUME
14,
DECEMBER
1964
infection must be continued. Ambulation is gradual during a seven to ten day hospitalization. PROGNOSIS In regard to the maternal and fetal prognosis based on the present series of cases, the following observations deserve emphasis. The maternal age ranged from 19 to Age: The factor of age alone appeared to 39 years. have no prognostic significance. Heart Failure: Severe heart failure was present in approximately 35 per cent of previously reported patients during pregnancy.’ In a single patient (Case 1) cardiac decompensation developed in the postpartum period, and the congestive failure was most likely related to the large Blalock-Taussig anastomosis. Routine medical therapy including bedrest, restricted sodium intake, digitalization and chlorothiazide effected a favorable response. Cyanotic Episodes: The occurrence of increased cyanosis, tachycardia and weakness in Case 5 is a recognized complication in tetralogy of Fallot and may result in syncope or death.“’ Hamilton8 and Kerr-9 report similar events which in the absence of hemorrhage most likely represent increased pulmonary infundibular obstruction with an augmented right to left The etiology of the increased infundishunt. bular obstruction has not been established; however, a relation to norepinephrine has been These episodes responded to oxysuggested.iO morphine and procaine gen and sedation; also have been recommended. The lowest peripheral Arterial Saturation: arterial saturation of 77 per cent (Case 4) was obtained in the most hemodynamically severe lesion with levels in the other 4 patients ranging from 86 to 90%. These values were obtained in the nonpregnant state. Since arterial saturation is a reflection of the right to left shunt, it is affected by the magnitude of the pulmonic stenosis, the blood volume and the peripheral vascular resistance. During pregnancy the blood volume and peripheral vascular resistance vary. The degree of pulmonary valvular stenosis is relatively fixed ; however, variation may occur in the infundibular area, as noted Based on these factors, a lower arterial above. saturation might occur during childbearing in a Nasal oxygen patient with tetralogy of Fallot. was administered during labor and delivery to improve the arterial saturation. Hematocrit: When the hematocrit was 65 per cent or higher in cyanotic mothers, Neil111
872
Jacob>
has reported a higher incidence of spontaneous abortion. In our patients the hematocrits ranged from 41 to 50 per cent with the exception of Case 4, in which the hematocrit was 60 per cent in early pregnancy and ranged from 50-52 during the first two trimesters. This patient was the only maternal fatality. Electrocardiogram: The electrocardiograms Cases (Fig. 1) are of diagnostic significance. 1, 2 and 3 have clockwise QRS loops in the frontal plane with mean QRS vectors of The latter 2 cases have + 180’ and + 195’. mean QRS vectors described in pulmonic stenosis with an associated endocardial cushion defecti However, Case 4 had a pentalogy of Fallot at autopsy, not true endocardial cushion defect. This diagnostic possibility remains in right precordial ST-T wave Case 5. The changes can be correlated with the right ventricular systolic pressures as noted in Table 1. Right ventricular systolic pressures of 88 (Case 3) and 92 (Case 5) mm. Hg result in positive T waves in lead Vi whereas right ventricular systolic pressures of 120 (Case 1) and 130 (Case 4) mm. Hg produce coved S-T segments and inverted T waves in lead Vr. Intermittent ST-T wave changes in lead Vi (Case 2) are in keeping with a right ventricular systolic pressure of 110 mm. Hg. Therefore, the mean QRS vector may suggest a more complex cardiac defect while the ST-T wave changes in the right precordial leads reflect the severity of the right ventricular pressure. Ckst Roe~~ge~ogram: The roentgenographic findings of right ventricular enlargement, a hypoplastic pulmonary artery segment and hypovascular peripheral pulmonary vasculature with an essentially normal transverse cardiac diameter are characteristic of a tetralogy Case 3 illustrates of Fallot (Cases 2, 3 and 5). a right aortic arch commonly encountered in The enlarged transverse carthis anomaly.13 diac diameters illustrated in Cases 1 and 4 are suggestive of an associated complication. Mortality: In this series there was 1 maternal death (Case 4) from pulmonary hemorrhage which occurred at 26 weeks of pregnancy in a patient with a severe pentalogy of Fallot. The nature of the bleeding suggested hemorrhage from the bronchial arterial collateral circulation, although a definite bleeding point was not established at autopsy. Pulmonary hemorrhage is a rare complication of this abnormality.i3 The fetal deaths were in Cases 1 and 4.
In Case 4 it was due to the antepartum death In Case 1 no cause for the fetal of the mother. death at 38 weeks of pregnancy was discovered. There was no apparent relation to the maternal hematocrit, oxygen saturation or prolonged labor. According to Mendelson, “. . , death may be attributed to intrapartum fetal hypoxia produced by the tetralogy itself and intensified by uterine contractions.“i Both fetal deaths were in females, and at autopsy neither fetus had a cardiovascular anomaly. Ojsjwing: The birth weights of the living infants ranged from 3 lb. 15 oz. to 5 lb. 6 oz. and averaged 4 lb. 5 oz. This substantiates the previous report i1 that infants born of cyanotic Among the living children mothers are small. were four males and one female who have shown no clinical evidence of congenital heart disease.
SUMMARYAND CONCLUSIONS Five patients with a tetralogy or pentalogy of Fallot who underwent seven pregnancies are presented. The significant clinical features and management are discussed with particular reference to both the maternal and fetal mortality and prognosis. It would therefore appear that (1) a history of increased cyanotic or syncopal episodes, (2) congestive heart failure, (3) a hematocrit above 60 per cent, (4) electrocardiographic changes of right ventricular strain (coved S-T segments and inverted T waves in leads VhR-VI), (5) roentgen evidence of an enlarged transverse cardiac diameter, (6) peripheral arterial saturation below 80 to 85 per cent and (7) a right ventricular pressure of 120 mm. Hg or greater are unfavorable prognostic signs. When several of these factors are present in the same patient they would contraindicate conception, and serious doubt is cast that an existing pregnancy could be carried to a successful termination. ACKNOWLEDGMENT The author is indebted to B. Butler, HN(W), USN, C. Lentz, HM3, USN and Miss J. Didio for their assistance in preparing this paper. REFERENCES 1.
MENDELSON,
p. 157. pany.
2.
C. L. Cardiac Disease in Pregnancy, Philadelphia, 1960. F. A. Davis Com-
DA FONSECA, J. M., FERRRISA, D. and DIAS, F. 0.
Gravidez associada a tetralogia de Fallot. C&i. Inst. Mutern., 12: 21, 1960. 3.
BAKER, J. L., et al.
Closed pulmonary
&v.
valvulotomy
THE AMERICAN JOURNAL OF CARDIOLOGY
Pregnancy
4.
5.
6.
7.
and Tetralogy
in the management of Fallot’s tetralogy complicated by pregnancy. J. Obst. B Gynaec. Brit. Emp., 70: 154, 1963. DIEMINGER, H. J. Schwangershaft und Geburt bei Fallotscher Tetralogie. Zentrabl. Gynaek., 83: 778, 1961. WOOD, P. Diseases of the Heart and Circulation, p. 427. Philadelphia, 1956. J. B. Lippincott Company. WOOD, P. Symposium on congenital heart disease; attacks of deeper cyanosis and loss of consciousness (syncope) in Fallot’s tetralogy. Brit. Heart J., 20: 282, 1958. BRAUDO, J. L. and ZION, M. M. The cyanotic $yn1~;~al&a~~tt~ in Fallot’s tetralogy. Brit. M.
9.
10.
11.
12.
13. 8. HA&TON, BI E. and THOMSON K. J. The heart in pregnancy and the childbeing age. In: Heart
VOLUME 14, DECEMBER1964
of Fallot
873
Disease and Pregnancy, p. 211. Edited by BURWELL, C. S. and METCALFE, J. Boston, 1958. Little Brown and Company. KERR, A., JR. and SODEMAN,W. A. Congenital heart disease in pregnancy. Am. Heart J., 42: 436,195l. JOHNSON,A. M. Norepinephrine and cyanotic attacks in Fallot’s tetralogy. Brit. Heart J., 23: 197, 1961. NEILL, C. A. and SWANSON,S. Outcome of pregnancy in congenital heart disease (Abstr.). Circulation, 24: 1003, 1961. SCOTT, L. P., HAUCK, A. J. and NADAS, A. S. Endocardial cushion defect with pulmonic stenosis. Circulation, 25: 653, 1962. TAUSSIG, H. B. Congenital Malformations of the Heart, Vol. II, pp. 31, 49. Cambridge, 1960. Harvard University Press.
and
Pentalogy of Fallot* COMMANDERWILLIAM J. JACOBY, JR., MC, usNt Portsmouth,
LTHOUGH persons with the abnormalities of Fallot probably constitute the largest group of patients with cyanotic congenital heart disease surviving to adulthood, little has appeared in the literature concerning pregnancy in this condition. The scant clinical experience in such patients was summarized by Mendelson’ who reviewed the 33 patients reported prior to 1959. Since that time reports of 8 patients have been added to the literature.24 The majority constitutesun operated cases; however, about 25 per cent have had palliative surgery including pulmonary valvulotomy or a shunt procedure. The lack of complete clinical and obstetric information prevents determination of the exact maternal and fetal mortality; however, Mendelson recorded 3 maternal and 11 fetal deaths with approximately one-third of the fetal losses resulting from interruption of pregnancies. The 5 cases in this paper, which represent form one of the largest seven pregnancies, single series reported to date. Based on the experience gained in caring for these patients the management and the significant factors affecting maternal and fetal prognosis will be discussed.
Virginia
and diminished cyanosis. In 1954 (age 16) a right frontal lobe abscess developed which was treated successfully by surgical drainage and antibiotics with little neurologic residual. From 1954 to 1959 she felt remarkably well. In March 1959 the patient was examined in the Maternity Cardiac Clinic, and the diagnosis of intrauterine pregnancy was estabAt this time her hematocrit was 46 per cent. lished. In April she was admitted to the hospital at 28 weeks of pregnancy during the peak blood volume period. Therapy included restricted activity and digitalization. In July (37 weeks) fluid retention developed but responded to restricted sodium diet and chlorothiazide. On July 13, (38 weeks) the patient went into labor. Nasal oxygen was administered and leg wraps applied. Fetal heart tones were recorded during labor; however, just prior to delivery none were audible, and she delivered a stillborn infant. In the early postpartum period, tachycardia, a ventricular gallop, bilateral basal rales and peripheral edema developed. She responded slowly to complete bedrest, digitalis, chlorothiazide and oxygen therapy. During the next two weeks gradual ambulation was permitted. She was discharged to the outpatient department one month postpartum. Physical Examination: The patient was thin and cyanotic. Blood pressure was 160/40-O mm. Hg. There were marked cervical pulsations bilaterally but no hepatojugular reflux. A healed left thoracotomy scar was present. The maximal palpable impulse was 12 cm. from the midsternal line in the fifth intercostal space. The rhythm was normal sinus. The first sound was increased and loudest at the apex. The second sound was of slightly increased intensity A third sound in diastole was noted at and unsplit. the apex along with an ejection sound at the left
A
CASE REPORTS CASE 1. A 21 year old white woman was noted to Dyspnea, squatbe cyanotic since shortly after birth. ting and retarded growth were present during childIn 1948 (age 10) a Blalock-Taussig shunr was hood. performed with marked symptomatic improvement
* From the Cardiology Branch (Department of Medicine) and Cardiopulmonary Laboratory, U. S. Naval Hospital, Portsmouth, Va. The opinions or assertions contained herein are those of the author and are not to be construed as official or reflecting the views of the Navy Department or of the Naval Service at large. i Present address: U. S. Naval Hospital, Great Lakes, Ill. 866
THE
AMERICAN JOURNALOF
CARDIOLOGY
Pregnancy Case 1
Case 2
and Tetralogy Case 3
of Fallot Case 4
867 case 5
aVR
aVL
aVF
FIG. 1. Electrocardiograms in the 5 cases. Note the direction of the QRS loop in the frontal plane and the variations in the mean QRS and ST-T wave vectors. VOLUME
14,
DECEMBER
1964
4
stt’rnal bordvr. .\ Sradc 3 (I h:~r\h ‘J *tolic LIIII~I~LU Mas heard along ~hc Icft ~tc‘rnal bordu. loudest in A diastolic the third and fortrth intercostal spaccq. ;2 rtunble follo\ted the third sound a~ the apex. grade 4 continuous murmur was audible in the second and third left intercostal spaces and transPeriphmitted to the intrascapular area posteriorly. eral pulses were full and the water-hammer type. The liver was palpable at the costal margin. No splenomegaly nor peripheral edema was present. The electrocardiogram (Fig. 1) demonstrated a first degree A-V block, right atria1 hypertrophy and right ventricular hypertrophy and strain. The roentgenogram of the chest (Fig. 2) demonstrated combined ventricular atria1 enlargement, right hypertrophy with the major ventricular involvement being most likely right-sided, increased peripheral pulmonav vasculature with the left pulmonary artery more prominent than the right, and a normal aortic configuration. The barium-filled esophagus was deviated posteriorly by the left atria1 enlargement. The cardiac catheterization data are presented in
‘l’abk I. .I‘he patient time of study.
5 was in mild heart failure at the
CME 2. l’his was a 39 year old white woman born of a normal pregnancy and delivery. A heart murmur and cyanosis were present at birth. Her growth and development were slightly retarded; however, she made adequate progress, suffering only easy fatigue, exertional dyspnea and increasing cyanosis with strenuous activity. At age 36 (1956) she had a full term, normal pregnancy with no complications. During the next two years the patient experienced some increasing dyspnea on exertion; however, she was able to perform her household duties. Cardiac catheterization was performed in 1958, and repeated in 1959. The patient’s second pregnancy occurred in 1959. Her management included bimonthly visits to the outpatient clinic until the last trimester when she was examined weekly. A restricted sodium diet was rigidly followed, and she required no oral diuretics. Her hematocrit was 58 per cent. She was admitted to the hospital in February 1960, two weeks prior to delivery, and her activity
Pregnancy
and Tetralogy
of Fallot
869
TABLE I Cardiac
Case
RA (mean)
Pressures--(mm. Hg) RV
PA
BA 130/llO/-
1 2
7 4
120/17 110/o
3u;;o
3
4
88/5
1415
4 5
4 4
130/O 92/P
14/5
.
Catheterization
Data
Dye Dilution Curve * (Shunt)
Arterial Saturation (a/o)
82/50
87
,..
77 a7
143190
L-R L-R R-L L-R R-L
86 90
L:R R-L
Angiocardiogram VSD PS (infundibular), PS (valvular and infundibular), VSD
PS, VSD
PS = pulmonic stenosis; VSD = ventricular septal defect; RA = right atrium; RV = right ventricle; PA = pulmonary artery; BA = brachial artery. * Dve dilution curves were uerformed with iniection into the RA except in Case 3 where injection was into the RV. Sampling was from the brachi’al artery. was markedly restricted. At the onset of labor, nasal oxygen was administered, and leg wraps were applied. The delivery was performed with meperidine analgesia, pudendal block and the application of low forceps. The infant was a full term, normal male, weighing 4 lbs. 8 oz. In the postpartum period she received prophylactic antiAmbulation was gradually increased during biotics. her two week hospitalization. Subsequent to her second pregnancy, the patient’s cardiovascular status had remained essentially unchanged. Physical Examination: The patient was well developed and slightly obese and demonstrated cyanosis of the lips, fingers and toes. Blood pressure was 130/90 mm. Hg. There was no cervical venous The lungs were clear. A systolic thrill distention. was palpable along the left upper sternal border and Normal sinus a right ventricular lift was present. The first sound was normal and rhythm was noted. loudest at the apex. A second sound was widely split with diminution of the pulmonary component. A grade 4 harsh systolic murmur was present over the entire precordium, loudest along the left sternal border in the second, third and fourth intercostal PeriphNo diastolic murmurs were audible. spaces. eral pulses were normal and equal bilaterally. There was no hepatosplenomegaly nor peripheral Mild clubbing of the fingers was noted. edema. The electrocardiogram (Fig. 1) showed right ventricular hypertrophy. The roentgenogram of the chest (Fig. 2) demonstrated a normal transverse cardiac diameter with prominent left pulmonary artery and diminished peripheral pulmonary vasculature. The cardiac catheterization findings were as noted in through an Table I. The catheter was manipulated atria1 septal defect from the right atrium to the left atrium and subsequently from the right ventricle to the aorta. VOLUME
14, DECEMBER
1964
CASE 3. This was a 20 year old Negro woman who gave a vague history; however, as a child she was At age 17 a murmur was noted treated for asthma. but not evaluated further until her first pregnancy in The initial pregnancy was uneventful from a 1960. cardiovascular standpoint; she delivered a full term normal infant. Her cardiac status remained unchanged, and cardiac catheterization was performed She had a second uneventful full term in July 1961. pregnancy in 1962. Instructions for routine outpatient visits and a restricted sodium intake were followed. No diuretics were required. Her hematocrit was 45 per cent. She had a spontaneous normal delivery with the infant weighing 5 lb. 6 oz. Physical Examination: The patient had mild cyanosis and clubbing of the fingers and toes. Blood pressure was 116/74 mm. Hg. No cervical venous distention was present. The lungs were clear. A systolic thrill was palpable in the third intercostal space along the left sternal border. The second sound was split with a diminished second component. A grade 3 systolic murmur was present over the precordium loudest along the left sternal border in the third intercostal space. No diastolic murmurs were Peripheral pulses were normal and equal. audible. There was no hepatosplenomegaly nor peripheral edema. The electrocardiogram (Fig. 1) demonstrated right ventricular hypertrophy. The roentgenogram of the chest (Fig. 2) illustrated right ventricular enlargement, slightly diminished pulmonary vasculature and the aorta descending on the right. The cardiac catheterization data are shown in Table I. A cineangiocardiogram was not obtained due to poor cooperation of the patient requiring termination of the study. CASE 4.
This was a 27 year old white woman born
Jacoby of a normal pregnancy and delivery. Shortly after her birth the family was informed that she had cyanotic congenital heart disease. During childhood frequent respiratory infections and squatting were denied but with increased activity she was easily fatigued and experienced dyspnea. Her cyanosis and clubbing remained unchanged. In 1956 a Her subsecardiac catheterization was performed. quent course had been uneventful except for the continued fatigue and dyspnea on exertion. On two occasions she had sharp anterior chest pain which was relieved by rest. This pain did not radiate to the arms or abdomen and was not associated with dyspnea, sweating or palpitations. She became pregnant During her twentieth week of pregnancy in 1961. she noted peripheral edema and was admitted to the hospital for further evaluation. Therapy included bedrest, restricted sodium diet and chlorothiazide. An initial response of weight loss ensued and then an An original uneventful hospitalization of five weeks. hematocrit was 60 per cent but was later maintained between 50 and 52 per cent. She was discharged to return in four days for rehospitalization. On the day of admission she experienced a sense of fullness in the throat followed by minimal hemoptysis. Coagulation studies were normal and the chest x-ray films remained unchanged from the initial films. During the next 48 hours minimal hemoptysis occurred. On the third hospital day massive pulmonary hemorrhage resulted in death. Physical Examination: The patient was moderately well developed and nourished and displayed diffuse cyanosis with no variation between the upper and lower extremities and equal clubbing of both fingers and toes. Blood pressure was 112/58 mm. Hg. The fundi revealed vascular engorgement and marked prominence of choroidal vessels. Auriculoventricular pulsations were present in the neck. There was slight prominence of the upper sternal region with a thrill palpable in the left second and third intercostal spaces. The lungs were clear. Normal sinus rhythm was present. The first sound was normal and loudest at the apex; the second, of slightly increased intensity, loudest at the third left intercostal space and with no evidence of splitting. An ejection sound was present along the left sternal border. A grade 3/6 harsh, systolic murmur was audible over the entire precordium but loudest in the second and third left intercostal spaces. No diastolic murmurs were audible. Peripheral pulses were normal and equal bilaterally. There was no hepatosplenomegaly nor peripheral edema. The electrocardiogram (Fig. 1) revealed right ventricular hypertrophy and strain. The roentgenogram of the chest (Fig. 2) showed dis-
first
sound
The
second
placement
unsplit,
of the heart
encroachment of right artery
in
the
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There
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The cardiac catheterization data are illustrated in Table I. Autopq: ‘I’he heart demonstrated pulmonary valvular (bicuspid valve) and infundibular stenosis, a ventricular septal defect, right ventricular hypertrophy and an atria1 septal defect. Pulmonary atelectasis and hemorrhage with a clot-cast of the bronchial tree was present. However, no specific area of hemorrhage or infarction was noted. There were no abnormal fetal cardiac defects. CASE 5. This was a 19 year old, white woman who had been aware of heart disease during her entire life and stated that she was a “blue baby.” During her early childhood her activity was restricted, and after exertion she was observed to squat. Her growth and development had been retarded, and she had suffered from multiple skeletal deformities. She had noted dyspnea on exertion and occasional increased cyanosis. In January 1961, she was admitted for cardiac catheterization. The patient was re-evaluated in December 1961, at which time she was 16 weeks pregnant. She tolerated her pregnancy well following a restricted sodium diet, and no diuretics were required. Her hematocrit was 50 per cent. During hospitalization from the twentysixth to the thirty-fourth her activity was severely restricted, and she was readmitted at 37 weeks of pregnancy. At the onset of labor, nasal oxygen was administered and leg wraps applied. An uncomplicated delivery was performed with meperidine analgesia, pudendal block and the application of low forceps. The infant weighed 4 lb. 3 oz. In the postpartum period several episodes of tachycardia, weakness and increased cyanosis with no loss of consciousness occurred. During these episodes sedation and an oxygen tent were employed with no other specific therapy. The attacks subsided, and ambulation was gradual. The patient was discharged in her third postpartum week. Physical Examination: The patient was short and moderately well nourished with obvious skeletal Blood pressure was 112/80 mm. Hg. deformities. The fundi were normal. There was no cervical A systolic thrill was palpable in venous distention. the suprasternal notch. The chest was deformed to the right by a thoracic scoliosis and associated lordosis which resulted in prominence of the right scapula and elevation of the right shoulder. The lungs were clear. A right ventricular lift and thrill were palpable along the right sternal border and midsternal
area
spaces.
A
and
in the second sinus was
to the fourth
arrhythmia normal
sound there
was
and
was
precordium,
loudest to the fourth
along
sternal
THE
border.
AMERICAN
the
intensity
with
spaces
No diastolic
JOURNAL
and
over the
midsternal
intercostal
The apex.
respiration.
was audible
in the
from the second the right
at
of increased
A grade 4 harsh systolic murmur entire
loudest
was no change
intercostal
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area and mur-
OF CARDIOLOGY
Pregnancy and Tetralogy of Fallot murs or gallops were audible. Peripheral pulses were normal and equal bilaterally. There was no There was cyanosis and clubhepatosplenomegaly. bing of the digits of the upper and lower extremities. A band constriction of the middle segment of the fourth left finger was present. The electrocardiogram (Fig. 1) demonstrated right ventricular hypertrophy. The roentgenogram of the chest (Fig. 2) revealed a thoracic scoliosis with a shift of the cardiac shadow to the right and right atria1 and right ventricular enlargement. The main pulmonary artery segment was not well visualized; however, there was slight prominence of the left pulmonary artery. The peripheral pulmonary vasculature was markedly reduced. The aorta was of normal configuration. The cardiac catheterization data are shown in Table I. An angiocardiogram was not performed. DISCUSSION Five cases of pregnancy in women with combined pulmonic stenosis, dextroposition of the aorta and a ventricular septal defect are presented. These lesions comprise the hemodynamitally significant malformations of a tetralogy of In two instances there was an associated Fallot. atria1 septal defect resulting in a pentalogy. Survival of these patients to the childbearing age is no longer unusual as evidenced by the increasing number of cases observed on large obstetrical services. In past decades cyanotic congenital heart disease was an indication for interruption of the pregnancy; however, this is not a valid concept since many of these patients will tolerate pregnancy under proper supervision. MANAGEMENT The medical management during childbearing must be stringent and uncompromising. Strict attention must be directed during frequent periodic outpatient visits to diet, weight gain, peripheral edema, anemia, cyanosis, syncope and changes in the physical examination or the electrocardiogram. Hospitalization during the period of peak blood volume and for one to two weeks prior to delivery may be necessary. Oral diuretics in conjunction with salt restriction are most useful in controlling At the time of delivery, leg fluid retention. prophylactic antibiotics and wraps, oxygen, Analavoidance of hypotension are indicated. gesia and pudendal block anesthesia at delivery were employed in the present series of patients. During the postpartum period the same careful attention to hypotension, anoxia, anemia and VOLUME
14,
DECEMBER
1964
infection must be continued. Ambulation is gradual during a seven to ten day hospitalization. PROGNOSIS In regard to the maternal and fetal prognosis based on the present series of cases, the following observations deserve emphasis. The maternal age ranged from 19 to Age: The factor of age alone appeared to 39 years. have no prognostic significance. Heart Failure: Severe heart failure was present in approximately 35 per cent of previously reported patients during pregnancy.’ In a single patient (Case 1) cardiac decompensation developed in the postpartum period, and the congestive failure was most likely related to the large Blalock-Taussig anastomosis. Routine medical therapy including bedrest, restricted sodium intake, digitalization and chlorothiazide effected a favorable response. Cyanotic Episodes: The occurrence of increased cyanosis, tachycardia and weakness in Case 5 is a recognized complication in tetralogy of Fallot and may result in syncope or death.“’ Hamilton8 and Kerr-9 report similar events which in the absence of hemorrhage most likely represent increased pulmonary infundibular obstruction with an augmented right to left The etiology of the increased infundishunt. bular obstruction has not been established; however, a relation to norepinephrine has been These episodes responded to oxysuggested.iO morphine and procaine gen and sedation; also have been recommended. The lowest peripheral Arterial Saturation: arterial saturation of 77 per cent (Case 4) was obtained in the most hemodynamically severe lesion with levels in the other 4 patients ranging from 86 to 90%. These values were obtained in the nonpregnant state. Since arterial saturation is a reflection of the right to left shunt, it is affected by the magnitude of the pulmonic stenosis, the blood volume and the peripheral vascular resistance. During pregnancy the blood volume and peripheral vascular resistance vary. The degree of pulmonary valvular stenosis is relatively fixed ; however, variation may occur in the infundibular area, as noted Based on these factors, a lower arterial above. saturation might occur during childbearing in a Nasal oxygen patient with tetralogy of Fallot. was administered during labor and delivery to improve the arterial saturation. Hematocrit: When the hematocrit was 65 per cent or higher in cyanotic mothers, Neil111
872
Jacob>
has reported a higher incidence of spontaneous abortion. In our patients the hematocrits ranged from 41 to 50 per cent with the exception of Case 4, in which the hematocrit was 60 per cent in early pregnancy and ranged from 50-52 during the first two trimesters. This patient was the only maternal fatality. Electrocardiogram: The electrocardiograms Cases (Fig. 1) are of diagnostic significance. 1, 2 and 3 have clockwise QRS loops in the frontal plane with mean QRS vectors of The latter 2 cases have + 180’ and + 195’. mean QRS vectors described in pulmonic stenosis with an associated endocardial cushion defecti However, Case 4 had a pentalogy of Fallot at autopsy, not true endocardial cushion defect. This diagnostic possibility remains in right precordial ST-T wave Case 5. The changes can be correlated with the right ventricular systolic pressures as noted in Table 1. Right ventricular systolic pressures of 88 (Case 3) and 92 (Case 5) mm. Hg result in positive T waves in lead Vi whereas right ventricular systolic pressures of 120 (Case 1) and 130 (Case 4) mm. Hg produce coved S-T segments and inverted T waves in lead Vr. Intermittent ST-T wave changes in lead Vi (Case 2) are in keeping with a right ventricular systolic pressure of 110 mm. Hg. Therefore, the mean QRS vector may suggest a more complex cardiac defect while the ST-T wave changes in the right precordial leads reflect the severity of the right ventricular pressure. Ckst Roe~~ge~ogram: The roentgenographic findings of right ventricular enlargement, a hypoplastic pulmonary artery segment and hypovascular peripheral pulmonary vasculature with an essentially normal transverse cardiac diameter are characteristic of a tetralogy Case 3 illustrates of Fallot (Cases 2, 3 and 5). a right aortic arch commonly encountered in The enlarged transverse carthis anomaly.13 diac diameters illustrated in Cases 1 and 4 are suggestive of an associated complication. Mortality: In this series there was 1 maternal death (Case 4) from pulmonary hemorrhage which occurred at 26 weeks of pregnancy in a patient with a severe pentalogy of Fallot. The nature of the bleeding suggested hemorrhage from the bronchial arterial collateral circulation, although a definite bleeding point was not established at autopsy. Pulmonary hemorrhage is a rare complication of this abnormality.i3 The fetal deaths were in Cases 1 and 4.
In Case 4 it was due to the antepartum death In Case 1 no cause for the fetal of the mother. death at 38 weeks of pregnancy was discovered. There was no apparent relation to the maternal hematocrit, oxygen saturation or prolonged labor. According to Mendelson, “. . , death may be attributed to intrapartum fetal hypoxia produced by the tetralogy itself and intensified by uterine contractions.“i Both fetal deaths were in females, and at autopsy neither fetus had a cardiovascular anomaly. Ojsjwing: The birth weights of the living infants ranged from 3 lb. 15 oz. to 5 lb. 6 oz. and averaged 4 lb. 5 oz. This substantiates the previous report i1 that infants born of cyanotic Among the living children mothers are small. were four males and one female who have shown no clinical evidence of congenital heart disease.
SUMMARYAND CONCLUSIONS Five patients with a tetralogy or pentalogy of Fallot who underwent seven pregnancies are presented. The significant clinical features and management are discussed with particular reference to both the maternal and fetal mortality and prognosis. It would therefore appear that (1) a history of increased cyanotic or syncopal episodes, (2) congestive heart failure, (3) a hematocrit above 60 per cent, (4) electrocardiographic changes of right ventricular strain (coved S-T segments and inverted T waves in leads VhR-VI), (5) roentgen evidence of an enlarged transverse cardiac diameter, (6) peripheral arterial saturation below 80 to 85 per cent and (7) a right ventricular pressure of 120 mm. Hg or greater are unfavorable prognostic signs. When several of these factors are present in the same patient they would contraindicate conception, and serious doubt is cast that an existing pregnancy could be carried to a successful termination. ACKNOWLEDGMENT The author is indebted to B. Butler, HN(W), USN, C. Lentz, HM3, USN and Miss J. Didio for their assistance in preparing this paper. REFERENCES 1.
MENDELSON,
p. 157. pany.
2.
C. L. Cardiac Disease in Pregnancy, Philadelphia, 1960. F. A. Davis Com-
DA FONSECA, J. M., FERRRISA, D. and DIAS, F. 0.
Gravidez associada a tetralogia de Fallot. C&i. Inst. Mutern., 12: 21, 1960. 3.
BAKER, J. L., et al.
Closed pulmonary
&v.
valvulotomy
THE AMERICAN JOURNAL OF CARDIOLOGY
Pregnancy
4.
5.
6.
7.
and Tetralogy
in the management of Fallot’s tetralogy complicated by pregnancy. J. Obst. B Gynaec. Brit. Emp., 70: 154, 1963. DIEMINGER, H. J. Schwangershaft und Geburt bei Fallotscher Tetralogie. Zentrabl. Gynaek., 83: 778, 1961. WOOD, P. Diseases of the Heart and Circulation, p. 427. Philadelphia, 1956. J. B. Lippincott Company. WOOD, P. Symposium on congenital heart disease; attacks of deeper cyanosis and loss of consciousness (syncope) in Fallot’s tetralogy. Brit. Heart J., 20: 282, 1958. BRAUDO, J. L. and ZION, M. M. The cyanotic $yn1~;~al&a~~tt~ in Fallot’s tetralogy. Brit. M.
9.
10.
11.
12.
13. 8. HA&TON, BI E. and THOMSON K. J. The heart in pregnancy and the childbeing age. In: Heart
VOLUME 14, DECEMBER1964
of Fallot
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Disease and Pregnancy, p. 211. Edited by BURWELL, C. S. and METCALFE, J. Boston, 1958. Little Brown and Company. KERR, A., JR. and SODEMAN,W. A. Congenital heart disease in pregnancy. Am. Heart J., 42: 436,195l. JOHNSON,A. M. Norepinephrine and cyanotic attacks in Fallot’s tetralogy. Brit. Heart J., 23: 197, 1961. NEILL, C. A. and SWANSON,S. Outcome of pregnancy in congenital heart disease (Abstr.). Circulation, 24: 1003, 1961. SCOTT, L. P., HAUCK, A. J. and NADAS, A. S. Endocardial cushion defect with pulmonic stenosis. Circulation, 25: 653, 1962. TAUSSIG, H. B. Congenital Malformations of the Heart, Vol. II, pp. 31, 49. Cambridge, 1960. Harvard University Press.