Ostium Primum

Albert Jackson, M.D., and Pauline E. Garber, M.D., Wadsworth, Kans.

While atria1 septal defects of the ostium secundum type are frequent, those of the ostium primum type are relatively rare.

It is our purpose to report an unusual case of ostium primum in a 62-year-old man who had, in addition, stenosis of the aortic valve, myocardial infarction, and puImonary and myocardial tuberculosis.

CASE REPORT

This 62-year-old white male farmer began to complain of shortness of breath in 1954, ap- proximately 3 years prior to his last admission to hospital and his death. During his first year of illness he was treated by his family physician with Mercuhydrin. In 1955, he was admitted to the V. A. Center because of shortness of breath. There was no history of rheumatic fever.

Physical examination on his first admission in 19.55, showed a well-nourished white man. The blood pressure was 180/110 mm. Hg. Circulation time with Decholin was 17 seconds, and with ether, 4 seconds. Venous pressure was 10 cm. of water. The heart was enlarged to the left. A systolic thrill was felt in the pulmonic area, and there was a loud, harsh, Grade 4 systolic murmur over the pulmonary area. The pulmonic second sound was markedly accentuated and slightly split. There was some suggestion of clubbing of the fingers and toes.

X-ray of chest showed right auricular, right ventricular, and left ventricular enlargement and hyperemic lung fields. The aorta was elongated and tortuous, with calcification in its arch. The ECG showed left axis deviation, right heart enlargement, and ventricular premature contrac- tions. A congenital heart disease, probably an atria1 septal defect, was considered, but the patient did not agree to cardiac catheterization. He was digitalized, with a satisfactory response, was discharged, and was well for 2 years. He was able to do farm work until a few weeks prior to his second admission when he again became dyspneic.

On his second admission in 1957, the patient was in extremis and markedly cyanotic. Moist rPles were heard over the lung fields. The liver extended to the umbilicus and was tender. There was massive edema. The heart sounds were very distant.

PostMortem Ejcaminui&nr.-The heart weighed 1,000 grams. The valve orifices measured as

follows: tricuspid 17 cm., pulmonic 10.5 cm., mitral 13 cm., and aortic 6 cm. The left ventricle was 1.5 mm. in thickness and the right measured as much as 13 mm. (Fig. 1). The heart showed marked enlargement, and the right ventricle and atrium were disproportionately increased in size. The left auricle was not enlarged. The myocardium was firm in consistency. Cut sections through the anterior septum revealed a few mottled an& hyperemic markings grossly producing the picture of a recent infarct.

From the Medical and Pathology Services of the Veterans Administration Center. Wadsworth, Kans.

Received for publication Dec. 27, 1957.

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Fig. l.-Note relative thickness of right and left ventricles.

Fig. 2.-Ostium primum, view from left ventricle.

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Fig. 3.-Ostium primum, view from right ventricle.

Fig. 4.-Stenotic aortic valve.

Located in the interatrial septum was a defect measuring 3.5 cm. in diameter. Anatomically, it lay just superior to the insertion of the mitral and tricuspid valves. The foramen ovale was anatomically closed and located above, and independent of, the defect. The mitral and tricuspid valves were intact and demonstrated no appreciable thickening. There was no cleft mitral or tricuspid valve present (Figs. 2 and 3). The aortic valve showed fusion of two of the cusps, with considerable deposition of calcium throughout producing a degree of rigidity and stenosis (Fig. 4).

The coronary arteries showed mild diffuse atheromatous changes, and at one level the lumen of the anterior descending branch was filled with a recent thrombus. A cut section through the region of the anterior septum revealed mottled and hyperemic markings, producing the picture of a recent infarction. The lungs showed throughout the parenchyma innumerable smal!, firm areas averaging 6 cm. in diameter, which on cut section showed mottled grayish scar tissue. The pulmonary vessels were large and free from thrombi.

Microscopic Examination.-Sections from the recent myocardial infarct showed characteristic coagulation necrosis. Also, located in the myocardium were small granulomas, consisting of lym- phocytes and giant cells, and a few epithelioid-like cells. No Aschoff bodies were demonstrated.

The lungs showed small tubercles consisting of epithelioid and giant cells. There were nu- merous lymphocytes present. Several acid-fast bacilli were demonstrated by Fite’s stain in one of the lymph nodes. The pulmonary vessels showed moderate sclerotic changes.

DISCUSSION

Ostium primum defect of the atria1 septum is usually, but not always, associated with incomplete division of atrioventricular canal. Some authors divide these conditions into two separate categories.’ Others consider this un- necessary, and include all these cases under the same caption of “ostium atrio- ventriculare commune.“2 All gradations can occur, from cases like ours with ostium primum without ventricular septal defect and intact A-V valves to a complete atrioventriculare commune.3s4

Longevity in patients with atria1 septal secundum type defects without as- sociated anomalies is not unusual.5 Longevity in cases of atria1 septal defects of the secundum type with Lutembacher’s syndrome,‘j and in cases of the ostium primum type with Lutembacher’s syndrome have been reported.7 Longevity in patients with ostium primum defects without associated anomalies is rather rare; usually such patients die in infancy.2

Association of ostium primum with Lutembacher’s syndrome with acquired syphilitic aortic insufficiency occurs infrequently.8sg However, to our knowledge, no case of ostium primum defect without Lutembacher’s syndrome and with an associated acquired calcific aortic stenosis has been reported.

The pulmonary tuberculosis was an accidental finding in our patient. The immediate cause of his death was the thrombosis of the coronary artery with myocardial infarction. Tuberculous myocarditis, which is rather rare,lO was also present in this patient, and it is quite possible that it might have been a contribut- ing factor in the fatal outcome of the myocardial infarction. Furthermore, this case is also proof that hyperemic lung fields do not always protect the patient from pulmonary tuberculosis.

It is amazing that the patient, with this large defect in the atria1 septum, had lived to the age of 62. Without any difficulty he had done heavy farm work all his life. As far as we could ascertain, no similar case has been described of a patient with ostium primum without cleft mitral valve, who lived to the age of

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62. Nor to our knowledge has there been any case report of a congenital atria1 septal defect of the ostium primum type with acquired aortic stenosis. It has to be postulated that those two lesions are purely coincidental.

SUMMARY

In summary, this 62-year-old man had an ostium primum, marked right ventricular hypertrophy, a calcific aortic stenosis, pulmonary and myocardial tuberculosis, and, as a final episode, a myocardial infarct.

REFERENCES

1. Von Rokitansky, C. F.: Die Defecte der Scheidewande des Herzens. mische Abhandlung, Wien, 1875, W. Braumuller.

Pathologisch-anato-

2. Rogers, H. M., and Edwards, J. E.: AM. HEART J. 36:28, 1948. 3. Blount, S. G., Balchum, 0. J., and Gensini, G.: Circulation 13:499, 1956. 4. Brandenburg, R. O., and DuShane, J. W.: Proc. Staff Meet. Mayo Clin. 31509, 1956’ 5. Stannus, D. G., Lansman, W., and Reed, F. A.: J. Florida M. A. 41:947, 1955. 6. Ellis, F. R., Greaves, M., and Hecht, H. H.: AM. HEART J. 40:154, 1950. 7. Askey, J. M., and Kahler, J. E.: Ann. Int. Med. 33:1031, 1950. 8. Lipson, M.: AM. HEART J. 35:497, 1948. 9. Kirshbaum, J. D., and Perlman, L.: Illinois M. J. 76:380, 1939.

10. Friedberg, Ch. K.: Diseases of the Heart, ed. 2, Philadelphia, 19.56, W. B. Saunders Com- pany, p. 913.