CASE REPORTS

* Combined Auricular Septal Defect and Thrombosis of the Major Pulmonary Artery * Spontaneous Perforation of the Esophagus * Cat Scratch Disease on the San Francisco Peninsula * Cat Scratch Disease in the Los Ahgeles Area

Combined Auricular Septal Defect and tions or petechiae. Moderate clubbing of the fingers Thrombosis of the Major Pulmonary Artery and toes was noted. Ocular movements, pupillary reactions, and visual fields were normal. There were PAUL CARNIG. M.D.. and HERBERT 1. HARDER, M.D., Glendale no hemorrhages or exudate in the retinae but the retinal veins were full and bluish in color. The disc IN A RECENT clinicopathological conference3 re- margins were well outlined. In the throat there was ported in the New England Journal of Medicine it cyanosis of the buccal mucosa. There was no ob- was stated that there have been only about five vious distention of the veins in the neck but the cases of combined auricular septal defect and major carotid pulsations were active. pulmonary artery thrombosis reported in the litera- "Fullness" in the right superior-anterior portion ture. Following is a report of a case in which those of the thorax and a slight inspiratory lag on the left conditions coexisted. side were noted. The lungs were resonant to percus- sion, and normal breath sounds were present on REPORT OF A CASE auscultation. The precordium was hyperactive. A palpable diastolic thrill was present in the third left A 30-year-old married Caucasian woman had al- interspace. The cardiac rhythm was regular. The ways been able to engage in strenuous athletic heart was considerably enlarged to the right and the activities (worked as a swimming instructor) with- pulmonary conus was prominent. The tones were out any symptoms until the age of 21, when she first partially obscured by a rather pronounced rumbling, noticed exertional dyspnea. She was then told that diastolic murmur in the second and third left inter- she had "a murmur." Dyspnea gradually increased, spaces. It was transmitted over the entire precordium and when the patient was 24 years of age cyanosis and was audible posteriorly in the interscapular and edema at the ankles were also noted. Digitalis area just to the left of the spine. A systolic murmur was given and a diet low in salt prescribed, but of lesser intensity was heard over the apex. P-2 was dyspnea gradually increased. At the age of 26 the accentuated over A-2. No friction rubs were present. patient had one episode of hemoptysis. From about The edge of the liver, firm but not tender and not that time onward, exertional syncope occurred oc- pulsating, was palpable 2 cm. below the right costal casionally and paresthesia of the extremities was margin. There was no dependent edema. The dor- frequently noticed, but there was no pulmonary salis pedis pulses were palpable and of good volume. edema. Upon neurological examination it was noted that The patient was hospitalized 17 days during July the cranial nerves were intact and that motor and 1950 for study. The temperature was 98 degrees F., sensory functions were normal. The sensorium was the pulse rate 72, respirations 28 per minute. The clear. blood pressure in the right arm was 115 mm. of The patient was given 0.1 mg. of digitoxin daily. mercury systolic and 70 mm. diastolic; in the left, Thiomerin® (mercaptomerin sodium) was given 118 mm. and 70 mm., respectively. The patient was intramuscularly as needed to control edema. On the very thin, cyanotic and in moderate respiratory dis- tenth hospital day cardiac catheterization was car- tress, with orthopnea developing even after the exer- ried out after prophylactic administration of penicil- tion of talking. The skin was generally pale, but cya- lin and procaine amide hydrochloride. Throughout nosis was present about the nose and mouth and the 24-hour period following catheterization, the under the nails. There was no jaundice and no erup- patient had very low blood pressure, as low as 50 From the Glendale Sanitarium and Hospital. mm. of mercury systolic and 30 mm. diastolic for 42 CALIFORNIA MEDICINE brief periods. However, there was good respontse to space. Examined fluoroscopically, the pulmonary symptomatic treatment and the patient was dis- conus and hilar vessels were observed to pulsate charged with prescription of digitoxin and occa- more than normally. sional injections of Thiomerin. Tenth day. Results of cardiac catheterization were: Oxygen Volumes in Laboratory Data Oxyl n Volumes ~-mmPressure.-mm.l. of Mercury- First hospital day. Venous pressure: 165 mm. of Catheter Position PerCent Systolic Diastolic Superior vena cava...... 10.97 .... blood, right antecubital space (No. 20 needle). Cir- Right auricle...... 11.83 14 6 Right ventricle (inflow tract) 12.15 37 23 culation time: 16 seconds, arm to tongue (Decholin). Right ventricle (outflow Vital capacity: 3.0 liters. Electrocardiogram: Auric- tract) ...... 11.50 40 10 ular rate 81; ventricular rate, 81; P-R interval, 0.16 Pulmonary artery (position 2)...... 11.8927 16 seconds; rhythm, sinus arrythmia; P wave in lead Pulmonary artery

V2 inverted; QRS complexes, pronounced right axis (position 3)...... 10.91.... Capacity ...... 25.09.... deviation, increased amplitude throughout, normal Femoral artery ...... 12.58 56 ~ii.... duration; T wave in leads 3 and V5 inverted; ST Saturation (femoral artery) 50.10 ...... segment in leads 2, 3, and V5 depressed; ST seg- Pulmonary vein (assumed as ment in leads V1 and V3 elevated. Interpretation of 95 per cent saturation) .... 24.09 .... Flow Indext electrocardiogram: Right axis deviation, right ven- 143 tricular "strain" and/or digitalis effect. *Systemic flow = = 121 78 The urine was yellow and clear with specific 12.58- 11.40 gravity 1.014 and pH 6.0. There was a trace of sugar. 143 *Pulmonary flow = = 11.26 7.31 In microscopic examination occasional leukocytes 24.09 11.40 were noted, but no erythrocytes or casts. Results of a phenolsulfonphthalein test were: It was felt at the time of catheterization that there was a large right-to-left shunt through an auricular Minutes Volume Per Cent defect, although the possibility of a ventricular sep- 30. 160 32 90.220 27 tal defect could not be ruled out. 120.50 8 The clinical impression was: Congenital heart dis- Total...... 67 ease, cyanotic type, with probable auricular septal defect, with a right-to-left shunt, accompanied by Second day. The erythrocyte content of the blood pulmonary hypertension. was 7.01 million per cu. mm. and the hemoglobin About a year later the patient was seen at home content 19.6 gm. per 100 cc. Leukocytes numbered because of an upper respiratory tract infection, for 8.150-polymorphonucleocytes 64 per cent, lympho- which penicillin was given. A few months later she cytes 27 per cent, and monocytes 9 per cent. Mean was again observed at home on complaint of "pleu- corpuscular volume, hemoglobin and hemoglobin ritic" pain. The blood pressure was 110 mm. of mer- concentration were within normal limits, and the cury systolic and 80 mm. diastolic. The thoracic pain erythrocyte sedimentation rate (not corrected for became more severe in the next week; then pro- the increased number of cells) was 1 mm. in one nounced dyspnea and acute pulmonary edema devel- hour. The icterus index was 6 units. Content of non- oped suddenly and the patient died. protein nitrogen was 39 mg. per 100 cc., of sugar 76 mg. per 100 cc., and of carbon dioxide 23.7 mEq. Autopsy The result of a test for blood in the stool was nega- Upon postmortem examination, pronounced cya- tive. nosis of the lips, face, upper trunk and nail beds was Third day. In an orthocardiogram the heart was noted and there was considerable livor of the depen- noted to be greatly enlarged in the right ventricular dent portions of the body and moderate clubbing of area. The left auricle did not appear to be enlarged. the fingers and toes. The heart weighed 520 gm. The view of the left ventricle was obscured by the (normal 250-300 gm.) and appeared enlarged in all shadow of the spine and it was felt that this posi- dimensions. The right side of the heart was greatly tion might be owing to enlargement of the left ven- dilated and hypertrophied; the right ventricle was tricle or-.to considerable displacement caused by the 18 cm. in circumference and the thickness 9 mm. The abnormal size of the right ventricle. The pulmonary The flow measurements are calculated by dividing the oxygen arterial hilar consumption of 143 cc. per minute by the arteriovenous differences. conus, pulmonary segment and vessels Thus: The arteriovenous difference for systemic flow is 12.58 (femoral were The vessels did not artery) minus 11.40 (mean content between superior' vena cava and huge. peripheral pulmonary right auricle) . For pulmonary flow it is: 24.09 (pulmonary vein) appear to be enlarged. The aorta was small and ap- minus 11.40 (mean auricular content). tThe cardiac index is the liters of blood flow per square meter of peared to be on the left. There was some fibrosis body surface area. It is calculated by dividi'gthe usual flow measure- ment by the surface area of the patient (in, the present case, 1.54 in the peripheral portion of the left second inter- square meters). VOL. 80. NO. 1 * JANUARY 1954 43 Patent ForormenOvole

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Figure 1.-Patent forarnen ovale of interatrial septum. sema and edema, severe congestion and an area of hemorrhagic infarction adjacent to the pulmonary left side of the heart was also enlarged, but this was artery. In the liver, distention of the sinusoid spaces apparently owing to dilation of the left ventricle was noted. Sections of other organs showed slight which was 12 cm. in circumference and 10 mm. thick. congestion or no significant lesion. Sectioned surfaces showed no evidence of old or recent infarction. All of the valves were competent SUMMARY and free of vegetation or any other evidence of dis- A case of auricular septal defect with almost com- ease. The interatrial septum had a patent foramen plete thrombotic occlusion of the main pulmonary ovale with a diameter of 5.1 cm. (Figure 1). The artery at its bifurcation is presented. No evidence of aorta was narrowed to a diameter of 1.3 cm. at a embolism or of underlying pulmonary artery disease point 4 cm. above the aortic valve. The coronary could be found. Cardiac catheterization 16 months arteries were normal. The pulmonary artery was considerably dilated and appeared to lead directly before death gave evidence of pulmonary hyperten- into the left lung. At the hilum, however, it bifur- sion and septal defect with a right-to-left shunt. The cated into the right and left branches. At the point condition of the patient deteriorated progressively of bifurcation the pulmonary artery was almost com- for nine years. She died of acute pulmonary edema pletely occluded by a thrombus showing prominent at the age of 30. lines of Zahn (Figure 2). The thrombus, which was firm and adherent, extended into both branches of ACKNOWLEDGEMENT the pulmonary artery and into their major branches. The authors are indebted to Edyth Schoenrich, M.D., for The lumen of these arteries was approximately 5 per the clinical data, and to Richard Bing, M.D., for data on cent of normal. There was 300 cc. of fluid in the left the cardiac catheterization, which was done at the Johns pleural space and 100 cc. in the right. The lungs felt Hopkins Hospital, Baltimore. firm and on cut surfaces showed edema and conges- 1509 East Wilson Avenue. tion with a 6 mm. area of apparent infarction near the hilum of the left lung. Severe congestion of the REFERENCES liver was noted. In other organs there were no sig- 1. Barber, J. MI., Magidson, O., and Wood, P.: Atrial nificant lesions. septal defect with special reference to electrocardiogram, pulmonary artery pressure and second heart sound, Brit. H. MicrOscopic examrination. Sections of the pulmo- J., 12:277, July 1950. nary artery were observed to be almost completely 2. Brannon, E. S., Weems, H. S., and Warren, J. V.: Atrial was septal defect-study of hemodynamics by the technique of occluded by a thrombus that well organized in right heart catheterization, Am. J. Med. Sci., 210:480, 1945. its deeper portions with platelet, fibrin and red-cell 3. Clinical Pathological Conference, N.E.J.M., 246:419, layering only in the superficial and surface portions. 1952. No abnormality was noted in the wall of the artery. 4. Hickam, J. B.: Atrial septal defect-a study of intra- cardiac shunts, ventricular outputs, and pulmonary pressure In sections of the heart slight hypertrophy of the gradient, Am. H. J., 38:801, Dec. 1949. myofibrillae composing the right ventricular wall 5. Irvin, G. E.: Contribution to the pathogenesis of chronic was noted but there was no other significant lesion. cor pulmonale-report of a case with multiple aneurysms, intravascular bands, and old massive thrombosis of the pul- Sections from both lungs showed moderate emphy- monary artery, Am. H. J., 37:1144, June 1949. 44 CALIFORNIA MEDICINE