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Congenital tricuspid atresia
CONGENITAL R. MAXWELL
ANDERSON,
TRICUSPID M.D.,*
ATRESIA
AND EDWARD
CHARLESTON,~.
E. MCKEE,
M.D.**
C.
recent reportsiJ have confirmed the rarity of the lesion and the SEVERAL brevity of life in congenital tricuspid atresia. Taussig3 stated that “most infants die before one year of age.” The oldest S-year-old child reported by Kiihne. 4 The following since the patient lived to an older age than any It is interesting that her supposedly identical twin
case appears to be that of a case is added to the literature previously reported patient. had no congenital anomalies.
CASE REPORT W. P., a 6-year-old white girl, was admitted to Roper Hospital on Nov. 6, 1950, because of convulsions. The child was one of twin girls delivered uneventfully and considered normal at birth. At 5 months of age cyanosis was first observed, and it was recognized that the patient had a heart murmur. Intermittent cyanosis had been present since, but general health was comparable to that of the normal twin except for retarded physical development evident mostly during the past year. Until that time, the twins were identical in all physical features, and cyanosis was the main helpful distinguishing characteristic. Ten days prior to admission, the patient was found lying on the floor in an extremely cyanotic and exhausted state. She was treated with oxygen and penicillin and responded promptly but two days later had a generalized convulsive seizure leaving her again in the clinical state previously described. Nausea and vomiting and a third convulsion followed, and the patient was transferred to Roper Hospital. Physical examination revealed a small but well-developed and well-nourished white girl who was lethargic but cooperative. Temperature was 100” F. There was moderate cyanosis of the mucous membranes and skin, and there was clubbing of the nail beds. The chest was clear. The heart was enlarged with the point of maximum apical intensity in the fifth intercostal space just outside the left mid-clavicular line. There was a loud, harsh Grade 3 systolic murmur heard over the entire precordium which was transmitted to the back and heard loudest in the third intercostal space to the left of the sternum. No thrills were palpable. There were no other murmurs. The liver edge was palpable 2 cm. below the right ccstal margin. An electrocardicgram revealed left axis deviation with left heart strain in a horizontal heart. The hemoglobin was 18 Gm., and the red blood count was 7 million. The white blood count was 17,500 with 87 per cent polymorphonuclear cells. Fluoroscopy of the heart revealed no definite chamber enlargement but a prominent pulmonary conus. The clinical impression was that this patient had a variant of the tetralogy of Fallot with probable tricuspid stenosis and underdevelopment of the right ventricle. It was felt that the convulsions were due to small cerebral thromboses secondary to hemoconcentration and cerebral anoxia. With cardiac surgery contemplated, catheterization of the right side of the heart was attempted on Nov. 8, 1950, after the patient had been placed on prophylactic penicillin at the time ---From the Medical College of the State of South Carolina, Charleston. Received for publication Sept. 27, 1851. *Prepared while Teaching Fellow in Cardiology, under joint rponsorshlp of the South Heart Association and the National Institutes of Health, United States Public Health Service. **Assistant Professor of Pathology of the Medical College of the State of South Carolina. 761
Carolina
762
AMERICAN
Fig l.-Persistent
Fig. 2.-Hypoplasia
ostium
of right
HEART
primum
ventricle
JOURNAL
(viewed
from
and interventricular
right
auricl .e).
septal
defed
ANDERSON AND MC KEE:
‘CONGENITAL
TRrcusPID ATRESIA
763
of admission. The catheter could be passed only a few centimeters into the antecubital vein of the left arm, and the procedure was discontinued because of extreme spasm of the veins. Penicillin was stopped the next day, and the temperature rose to 101.6” F. The patient remained lethargic and ancrexic, and penicillin was restarted. On Nov. 15, 1950, a blood culture was reported as growing nonhemolytic streptococci. It was now felt that the patient had subacute bacterial endocarditis. On Nov. 18, 19.50, the temperature rose to 106’ F., accompanied by a generalized convulsive seizure. Papilledema was observed for the first time. Respirations ceased, but life was maintained by means of a respirator with electrocardiograms showing normal sinus rhythm for some ten hours before cardiac action ceased. At time of death, it was felt that vegetations in the heart had thrown off septic emboli to the brain with development of a brain abscess.
Fig. 3.-Interventricular
septal defect (viewed from left ventricle).
Autopsy findings revealed a large brain abscess in the frontoparietal region, atresia of the tricuspid valve, but no evidence of subacute bacterial endocarditis. Culture from the brain abscess showed Pseudomonas aeruginosa. The heart weighed 100 grams and showed smooth, glistening endocardial surfaces. There was an interauricular septal defect, apparently ostium primum, which measured 1 by 0.6 cm. (Fig. 1). There was no direct communication between the right auricle and the right ventricle. The right ventricle was a roughly tubular shaped chamber 4 cm. in length and 1.2 cm. in diameter (Fig. 2). Its wall varied from 0.1 to 0.2 cm. in thickness. The circumference of the pulmonary valve measured 3.5 cm. The left ventricle measured 5 by 2 cm., and the myocardium was 0.8 cm. in thickness. The aortic valve ring was 3.5 cm. in circumference. Just inferior to the aortic ring there was a slitlike opening in the interventricular septum 1 cm. in length and less than 0.2 cm. in greatest width which communicated with the right ventricle (Fig. 3). Except for the absence of the tricuspid valve, no valvular defects were noted. The intima of the pulmonary artery and aorta showed surface dimpling at the level of the ductus arteriosus, but this structure had been obliterated and closed.
764
AMERICAN
HEART
JOURNAL
REFERENCES
1. Somm;yilS.
C., and Johnson, J. M.:
Congenital
Tricuspid
Atresia, AM. HEART
J. 41:130,
2. Kroop, I. G.: Congenital Tricuspid Atresia, AM. HEART J. 41:549, 1951. 3. Taussig, Helen B.: Congenital Malformations of the Heart, New York, 1947, Commonwealth Fund, p. 97. 4. Ktihne, Marie: Ueber zwei Falle kongenitaler Atresie des Ostium venosum dextrum, Jahrb. f. Kinderh. 63:235, 1906.
ANDERSON,
TRICUSPID M.D.,*
ATRESIA
AND EDWARD
CHARLESTON,~.
E. MCKEE,
M.D.**
C.
recent reportsiJ have confirmed the rarity of the lesion and the SEVERAL brevity of life in congenital tricuspid atresia. Taussig3 stated that “most infants die before one year of age.” The oldest S-year-old child reported by Kiihne. 4 The following since the patient lived to an older age than any It is interesting that her supposedly identical twin
case appears to be that of a case is added to the literature previously reported patient. had no congenital anomalies.
CASE REPORT W. P., a 6-year-old white girl, was admitted to Roper Hospital on Nov. 6, 1950, because of convulsions. The child was one of twin girls delivered uneventfully and considered normal at birth. At 5 months of age cyanosis was first observed, and it was recognized that the patient had a heart murmur. Intermittent cyanosis had been present since, but general health was comparable to that of the normal twin except for retarded physical development evident mostly during the past year. Until that time, the twins were identical in all physical features, and cyanosis was the main helpful distinguishing characteristic. Ten days prior to admission, the patient was found lying on the floor in an extremely cyanotic and exhausted state. She was treated with oxygen and penicillin and responded promptly but two days later had a generalized convulsive seizure leaving her again in the clinical state previously described. Nausea and vomiting and a third convulsion followed, and the patient was transferred to Roper Hospital. Physical examination revealed a small but well-developed and well-nourished white girl who was lethargic but cooperative. Temperature was 100” F. There was moderate cyanosis of the mucous membranes and skin, and there was clubbing of the nail beds. The chest was clear. The heart was enlarged with the point of maximum apical intensity in the fifth intercostal space just outside the left mid-clavicular line. There was a loud, harsh Grade 3 systolic murmur heard over the entire precordium which was transmitted to the back and heard loudest in the third intercostal space to the left of the sternum. No thrills were palpable. There were no other murmurs. The liver edge was palpable 2 cm. below the right ccstal margin. An electrocardicgram revealed left axis deviation with left heart strain in a horizontal heart. The hemoglobin was 18 Gm., and the red blood count was 7 million. The white blood count was 17,500 with 87 per cent polymorphonuclear cells. Fluoroscopy of the heart revealed no definite chamber enlargement but a prominent pulmonary conus. The clinical impression was that this patient had a variant of the tetralogy of Fallot with probable tricuspid stenosis and underdevelopment of the right ventricle. It was felt that the convulsions were due to small cerebral thromboses secondary to hemoconcentration and cerebral anoxia. With cardiac surgery contemplated, catheterization of the right side of the heart was attempted on Nov. 8, 1950, after the patient had been placed on prophylactic penicillin at the time ---From the Medical College of the State of South Carolina, Charleston. Received for publication Sept. 27, 1851. *Prepared while Teaching Fellow in Cardiology, under joint rponsorshlp of the South Heart Association and the National Institutes of Health, United States Public Health Service. **Assistant Professor of Pathology of the Medical College of the State of South Carolina. 761
Carolina
762
AMERICAN
Fig l.-Persistent
Fig. 2.-Hypoplasia
ostium
of right
HEART
primum
ventricle
JOURNAL
(viewed
from
and interventricular
right
auricl .e).
septal
defed
ANDERSON AND MC KEE:
‘CONGENITAL
TRrcusPID ATRESIA
763
of admission. The catheter could be passed only a few centimeters into the antecubital vein of the left arm, and the procedure was discontinued because of extreme spasm of the veins. Penicillin was stopped the next day, and the temperature rose to 101.6” F. The patient remained lethargic and ancrexic, and penicillin was restarted. On Nov. 15, 1950, a blood culture was reported as growing nonhemolytic streptococci. It was now felt that the patient had subacute bacterial endocarditis. On Nov. 18, 19.50, the temperature rose to 106’ F., accompanied by a generalized convulsive seizure. Papilledema was observed for the first time. Respirations ceased, but life was maintained by means of a respirator with electrocardiograms showing normal sinus rhythm for some ten hours before cardiac action ceased. At time of death, it was felt that vegetations in the heart had thrown off septic emboli to the brain with development of a brain abscess.
Fig. 3.-Interventricular
septal defect (viewed from left ventricle).
Autopsy findings revealed a large brain abscess in the frontoparietal region, atresia of the tricuspid valve, but no evidence of subacute bacterial endocarditis. Culture from the brain abscess showed Pseudomonas aeruginosa. The heart weighed 100 grams and showed smooth, glistening endocardial surfaces. There was an interauricular septal defect, apparently ostium primum, which measured 1 by 0.6 cm. (Fig. 1). There was no direct communication between the right auricle and the right ventricle. The right ventricle was a roughly tubular shaped chamber 4 cm. in length and 1.2 cm. in diameter (Fig. 2). Its wall varied from 0.1 to 0.2 cm. in thickness. The circumference of the pulmonary valve measured 3.5 cm. The left ventricle measured 5 by 2 cm., and the myocardium was 0.8 cm. in thickness. The aortic valve ring was 3.5 cm. in circumference. Just inferior to the aortic ring there was a slitlike opening in the interventricular septum 1 cm. in length and less than 0.2 cm. in greatest width which communicated with the right ventricle (Fig. 3). Except for the absence of the tricuspid valve, no valvular defects were noted. The intima of the pulmonary artery and aorta showed surface dimpling at the level of the ductus arteriosus, but this structure had been obliterated and closed.
764
AMERICAN
HEART
JOURNAL
REFERENCES
1. Somm;yilS.
C., and Johnson, J. M.:
Congenital
Tricuspid
Atresia, AM. HEART
J. 41:130,
2. Kroop, I. G.: Congenital Tricuspid Atresia, AM. HEART J. 41:549, 1951. 3. Taussig, Helen B.: Congenital Malformations of the Heart, New York, 1947, Commonwealth Fund, p. 97. 4. Ktihne, Marie: Ueber zwei Falle kongenitaler Atresie des Ostium venosum dextrum, Jahrb. f. Kinderh. 63:235, 1906.