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Absence of proximal coronary arteries associated with pulmonary atresia
596
Brief
Seplember, 1983 American Heart Journal
Communications
showed a faintly staining gram-negative cocci bacillus which grew on chocolate and Brucella agar in the presence of CO, in 5 days. Exposure to lead acetate produced H,S in 4 days. This organism was oxidase negative, catalase positive, and indole negative. Additional subcultures on specific media for gram-negative bacilli (Maconkey, Eosin-methylene blue A) were negative. This identified a member of the Brucella speciesas the organism causing infective endocarditis and confirmed the serologicdiagnosis. There were no postoperative complications, and the hospital course remained uneventful. The patient was discharged following the completion of a full 5-week courseof antibiotic therapy. One year following discharge the patient had gained 6 kg, was asymptomatic, and afebrile. The hemoglobin had risen to 13 gm/dl, and the ESR had dropped to 10mm the first hour. The Brucella titer had decreasedto 1: 800.There wasno evidence of splenomegaly. The aortic prosthetic valve sounds were normal. The left ventricular sizehad markedly decreased,both by clinical examination and by chestx-ray examination. The presenceof an active Brucella infection was diagnosedpreoperatively, basedon the rising serum antibody titers. In view of the clinical findings, the echocardiogram supported the diagnosisof Brucella infective endocarditis by the demonstration of the aortic valve vegetations. The lack of positive blood cultures may be explained by the fact that the patient had received numerous antibiotics prior to his admission;thus this finding wasnot completely unexpected. Nevertheless, postoperative bacteriologic studiesconfirmed the preoperative diagnosis.Traditionally, a combination of tetracycline and streptomycin has beenusedin the treatment of Brucella infections. The use of trimethoprim-sulfamethoxazole hasbeen proven to be a useful antibiotic2s5regimen in the treatment of Brucella infections, with the advantage of decreasedtoxicity when compared to the tetracycline-streptomycin regime over a period of prolonged administration. Since our patient had received various antibiotic therapies for an unknown time period prior to admission and the increased BUN and creatinine levels suggesteda moderate restriction of his renal function, it waselected to treat him with intravenous trimethoprim-sulfamethoxazole. Replacement of the aortic valve was necessarybecauseof the severe refractory congestive heart failure. The aggressivesurgical management ha9 been shown to decrease patient mortality in selectedcasesof Brucella endocarditis.4zfiAlthough rheumatic valcular diseaseis not a prerequisite for infective endocarditis, it is noteworthy that our patient had the pathologic findings of healed rheumatic disease,which may have contributed to the subsequentdevelopment and destruction of the aortic valve leaflets by the Brucella organisms.
Wray TM: The variable echocardiographic features in aortic valve endocarditis. Circulation 52:658,- 1975. Cleveland J. Suchar R. Daehe J: Destructive aortic valve endocarditis’from Brucella aYbortus: Survival with emergency aortic valve replacement. Thorax 33:616, 1978. Hassan A, Erian MM, Farid Z, Hathout SD, Sorensen, K: Trimethoprim-sulfamethoxazole in acute brucellosis. Br Med J 3:159, 1971. Parrott JC, Hill JD, Kerth WJ, Gerbode F: The surgical management of bacterial endocarditis: A review, Ann Surg 183:286,
1976.
Absence of proximal coronary arteries associated with pulmonary atresia Ken Ueda, M.D., Akihiro Saito, M.D., Hiroyuki Nakano, M.D., and Yutaka Hamazaki, M.D. Shizuoka,
Japan
Congenital malformation9 of coronary arteries are relatively uncommon, especially absence of the proximal coronary arteries, first reported by Lenox and Briner,’ which is extremely rare. An infant is describedherein with postmortem findings of no communications between the coronary arteries and the aorta or pulmonary artery, associatedwith severetricuspid stenosis,pulmonary atresia, and patent ductus arteriosus. A 2-day-old male infant was admitted to Shizuoka Children’s Hospital becauseof generalized cyanosis and respiratory failure. He was born of an uncomplicated pregnancy and delivery, and weighed3300gm at birth. On admission,he wastachypneic and severely cyanotic. There wasno cardiac murmur or pulmonary rales; the liver was enlarged. The chest roentogenogram revealed decreased pulmonary blood flow and the ECG demonstrated marked ST depressionin leadsII, III, aV, and V,, (Fig. 1). Cardiac enzymeswere not elevated. The infant died of intractable heart
failure
and arrhythmias
at 4 days of age, despite
treatment with digitalis, isoproterenol, diuretics, and other supportive measures. Cardiac malformations at autopsy consisted of severe stenosis of the tricuspid valve (pinhole), pulmonary atresia, marked hypoplasia of the right ventricle, patent ductus arteriosus, and anomaliesof the coronary arteries (Figs. 2 to 4). Three coronary arteries were connected to the hypoplastic right ventricle and were not connected to either the aorta or pulmonary artery. A coronary ostium or dimple was not demonstrated in any aortic sinus (Fig. 5). The first upper channel supplied the left main and circumflex arteries, which had almost normal branching (Fig. 2). The secondupper channel supplied a small right coronary artery (Fig. 3), and the third lower channel was
REFERENCES
1. Dorney RR: Endocarditis. In Hurst JW, and Logue RB, editors: The heart. New York, 1977, McGraw-Hill Book Co, Inc, p 1497. 2. Daikos GK, Papolyzos N, Marketos N, Mochlas S, Kastanakis S. Panasteriadis E: Trimethonrim-sulfamethoxazole in brucellosis. J Infect Dis 128(suppi):731, 1973.
From the Departments of Pediatric Cardiology and Pathology, Shizuoka Children’s Hospital. Reprint requests: Ken Ueda, M.D., Department of Pediatric Cardiology, Shizuqka Children’s Hospital, Urushiyama 860, Shizuoka-shi, 420 Japan.
Volume
106
Number
3
Brief
.I’: Fig.
11
\i
Communications
aVR
a%
aVF
s4
!‘j
1’6
5%~
1. Electrocardiogram demonstratesmarked ST depressionin leadsII, III, aVr, and V,.,.
Fig. 2. Fistulous connection between the left upper channel, which suppliesthe left anterior descendingcoronary and circumflex coronary arteries and the hypoplastic right ventricle is demonstrated. The pulmonary artery shows marked hypoplasia and the endocardium of the left ventricle does not demonstrate ischemic changes macroscopically. PA = pulmonary artery: Ao = aorta; RV = right ventricle; LV = left ventricle.
connected to short apical arteries (Fig. 4). The cut surface of the left ventricle did not show an area of myocardial infarction, and microscopic examination revealed no ischemic changes in the myocardium and subendocardium. Our patient represents the second instance of absent proximal coronary arteries with unique cardiocoronary fistulas arising from the hypoplastic right ventricle. The first case reported by Lenox and Brine9 resemblesour
Fig. 3. Fistulous connection between the right upper channel and the hypoplastic right ventricle is demonstrated. Arrow showsthe left upper channel. RV = right ventricle.
case,except that their casehad no stenosisof the tricuspid valve. In these two cases,there are common demerits concerning the coronary circulation. First, only systemic venous blood is available to the coronary circulation. Second, the coronary blood flow is not sufficiently sustained because of the direct connection between the coronary arteries and the right ventricle. Moreover, severe stenosisof the tricuspid valve in our caseobstructs inflow of blood to the right ventricle. Unfavorable coronary circulation due to these associated cardiac anomalies probably shortened survival to 4 days, in contrast with 2 months in the other reported case.’Absenceof myocardial and subendocardial ischemic changes by microscopic
598
Brief
September, 1983 American Heart Journal
Communications
Combined phenylephrine and tranylcypromine for postural hypotension Harold D. Itskovitz, M.D., and Alan Wartenburg, M.D. Milwaukee,
Fig. 4. Fistulous connection between the lower channel, which suppliesthe short apical coronary arteries, and the hypoplastic right ventricle is demonstrated. Ao = aorta; R V = right ventricle; L V = left ventricle; PA = pulmonary artery.
Fig. 5. Coronary ostium or dimple wasnot demonstrated in any aortic sinus. Ao = aorta; LV = left ventricle.
examination in the present caseis probably due to the shorter survival period. Embryogenesis of this lesion is very interesting but is by no meansclear. It is reasonable to consider the multiple fistulous communications between the right ventricle and the coronary arteries in this heart as an enlargement and persistence of the arteriosinusoidal and arterioluminal connections, which are well established before development of the coronary circulation.’ REFERENCES
1. Lenox CC, Briner J: Absent proximal coronary arteries associated with pulmonary atresia. Am J Cardiol 30~666, 1972. 2. Goor DA, Lillehei CW: Congenital malformations of the heart. New York, San Francisco, London, 1975, Grune & Stratton, Inc, p 84.
Wise.
Orthostatic hypotension is difficult to treat and many patients with this condition are severely disabled for life. We observed a female with postural hypotension (40/O mm Hg) and syncope following an acute viral infection. In addition, shedeveloped a paralytic bladder, absent sweating, and no pulsechangefollowing the Vdsalva maneuver. Her 24-hour urinary excretion of norepinephrine was minimal. Total blood volume wasnormal and there wasno laboratory evidence of abnormal pituitary, adrenal, or cardiac function to explain the patient’s postural hypotension. Our evaluation pointed to sympathetic nervous dysfunction. She was treated with intravenous saline, 9 alpha-fludrocortisone, indomethacin,’ and elastic stockings without relief of symptoms or increase in standing blood pressure(BP). Phenylephrine (4 mg/ml) by mouth to a doseof 30 mg every 4 hours gave inconsistent results and occasionally causedsupine hypertension with orthostatic hypotension. Treatment with the monoamine oxidase inhibitor (MAOI), tranylcypromine (10 mg twice daily), plus a high tyramine diet had no effect. However, when oral phenylephrine (10 to 15mg every 4-6 hours) was combined with tranylcypromine (10 mg twice daily), her supine BP reached 140 to 160/100 mm Hg and her standing systolic BP increased to 120 mm Hg. Pressor responsesoccurred 20 to 30 minutes after ingestion of phenylephrine and lasted 4 to 6 hours. With this regimen the patient’s BP wascontrolled for 6 months with few dose adjustments. Similar therapy was used successfully in three other patients. Dosesof phenylephrine ranged from 8 to 15 mg, four times daily. MAOIs have been prescribed for treatment of orthostatic hypotension with variable success2sJGenerally, these are usedwith ephedrine, tyramine, or foods high in tyramine. MAOIs benefit postural hypotension by decreasing degradation of norepinephrine and epinephrine to increasecirculating levels of endogenousvasoconstrictors. Also, they inhibit MAO in the gut and liver to allow greater gastrointestinal absorption of ingestedtyramine.4The tyramine increasesBP indirectly by enhancing the neural releaseof norepinephrine. Similarly, ephedrine releasesnorepinephrine, contributing in part to its hypertensive effect. Also, ephedrine is a beta-receptor agonist which increasesBP by increasing cardiac output. In each of these cases,vasoconstrictor effects depend upon the neural releaseof norepinephrine. However, drugs which act by releasing norepinephrine may be ineffective in patients with sympathetic lesions if norepinephrine is From
the Department
Reprint Medical
requests: College,
Harold Valhalla,
of Medicine, D. Itskovitz, NY 10595.
Medical M.D.,
College Munger
of Wisconsin. Pavilion,
New
York
Brief
Seplember, 1983 American Heart Journal
Communications
showed a faintly staining gram-negative cocci bacillus which grew on chocolate and Brucella agar in the presence of CO, in 5 days. Exposure to lead acetate produced H,S in 4 days. This organism was oxidase negative, catalase positive, and indole negative. Additional subcultures on specific media for gram-negative bacilli (Maconkey, Eosin-methylene blue A) were negative. This identified a member of the Brucella speciesas the organism causing infective endocarditis and confirmed the serologicdiagnosis. There were no postoperative complications, and the hospital course remained uneventful. The patient was discharged following the completion of a full 5-week courseof antibiotic therapy. One year following discharge the patient had gained 6 kg, was asymptomatic, and afebrile. The hemoglobin had risen to 13 gm/dl, and the ESR had dropped to 10mm the first hour. The Brucella titer had decreasedto 1: 800.There wasno evidence of splenomegaly. The aortic prosthetic valve sounds were normal. The left ventricular sizehad markedly decreased,both by clinical examination and by chestx-ray examination. The presenceof an active Brucella infection was diagnosedpreoperatively, basedon the rising serum antibody titers. In view of the clinical findings, the echocardiogram supported the diagnosisof Brucella infective endocarditis by the demonstration of the aortic valve vegetations. The lack of positive blood cultures may be explained by the fact that the patient had received numerous antibiotics prior to his admission;thus this finding wasnot completely unexpected. Nevertheless, postoperative bacteriologic studiesconfirmed the preoperative diagnosis.Traditionally, a combination of tetracycline and streptomycin has beenusedin the treatment of Brucella infections. The use of trimethoprim-sulfamethoxazole hasbeen proven to be a useful antibiotic2s5regimen in the treatment of Brucella infections, with the advantage of decreasedtoxicity when compared to the tetracycline-streptomycin regime over a period of prolonged administration. Since our patient had received various antibiotic therapies for an unknown time period prior to admission and the increased BUN and creatinine levels suggesteda moderate restriction of his renal function, it waselected to treat him with intravenous trimethoprim-sulfamethoxazole. Replacement of the aortic valve was necessarybecauseof the severe refractory congestive heart failure. The aggressivesurgical management ha9 been shown to decrease patient mortality in selectedcasesof Brucella endocarditis.4zfiAlthough rheumatic valcular diseaseis not a prerequisite for infective endocarditis, it is noteworthy that our patient had the pathologic findings of healed rheumatic disease,which may have contributed to the subsequentdevelopment and destruction of the aortic valve leaflets by the Brucella organisms.
Wray TM: The variable echocardiographic features in aortic valve endocarditis. Circulation 52:658,- 1975. Cleveland J. Suchar R. Daehe J: Destructive aortic valve endocarditis’from Brucella aYbortus: Survival with emergency aortic valve replacement. Thorax 33:616, 1978. Hassan A, Erian MM, Farid Z, Hathout SD, Sorensen, K: Trimethoprim-sulfamethoxazole in acute brucellosis. Br Med J 3:159, 1971. Parrott JC, Hill JD, Kerth WJ, Gerbode F: The surgical management of bacterial endocarditis: A review, Ann Surg 183:286,
1976.
Absence of proximal coronary arteries associated with pulmonary atresia Ken Ueda, M.D., Akihiro Saito, M.D., Hiroyuki Nakano, M.D., and Yutaka Hamazaki, M.D. Shizuoka,
Japan
Congenital malformation9 of coronary arteries are relatively uncommon, especially absence of the proximal coronary arteries, first reported by Lenox and Briner,’ which is extremely rare. An infant is describedherein with postmortem findings of no communications between the coronary arteries and the aorta or pulmonary artery, associatedwith severetricuspid stenosis,pulmonary atresia, and patent ductus arteriosus. A 2-day-old male infant was admitted to Shizuoka Children’s Hospital becauseof generalized cyanosis and respiratory failure. He was born of an uncomplicated pregnancy and delivery, and weighed3300gm at birth. On admission,he wastachypneic and severely cyanotic. There wasno cardiac murmur or pulmonary rales; the liver was enlarged. The chest roentogenogram revealed decreased pulmonary blood flow and the ECG demonstrated marked ST depressionin leadsII, III, aV, and V,, (Fig. 1). Cardiac enzymeswere not elevated. The infant died of intractable heart
failure
and arrhythmias
at 4 days of age, despite
treatment with digitalis, isoproterenol, diuretics, and other supportive measures. Cardiac malformations at autopsy consisted of severe stenosis of the tricuspid valve (pinhole), pulmonary atresia, marked hypoplasia of the right ventricle, patent ductus arteriosus, and anomaliesof the coronary arteries (Figs. 2 to 4). Three coronary arteries were connected to the hypoplastic right ventricle and were not connected to either the aorta or pulmonary artery. A coronary ostium or dimple was not demonstrated in any aortic sinus (Fig. 5). The first upper channel supplied the left main and circumflex arteries, which had almost normal branching (Fig. 2). The secondupper channel supplied a small right coronary artery (Fig. 3), and the third lower channel was
REFERENCES
1. Dorney RR: Endocarditis. In Hurst JW, and Logue RB, editors: The heart. New York, 1977, McGraw-Hill Book Co, Inc, p 1497. 2. Daikos GK, Papolyzos N, Marketos N, Mochlas S, Kastanakis S. Panasteriadis E: Trimethonrim-sulfamethoxazole in brucellosis. J Infect Dis 128(suppi):731, 1973.
From the Departments of Pediatric Cardiology and Pathology, Shizuoka Children’s Hospital. Reprint requests: Ken Ueda, M.D., Department of Pediatric Cardiology, Shizuqka Children’s Hospital, Urushiyama 860, Shizuoka-shi, 420 Japan.
Volume
106
Number
3
Brief
.I’: Fig.
11
\i
Communications
aVR
a%
aVF
s4
!‘j
1’6
5%~
1. Electrocardiogram demonstratesmarked ST depressionin leadsII, III, aVr, and V,.,.
Fig. 2. Fistulous connection between the left upper channel, which suppliesthe left anterior descendingcoronary and circumflex coronary arteries and the hypoplastic right ventricle is demonstrated. The pulmonary artery shows marked hypoplasia and the endocardium of the left ventricle does not demonstrate ischemic changes macroscopically. PA = pulmonary artery: Ao = aorta; RV = right ventricle; LV = left ventricle.
connected to short apical arteries (Fig. 4). The cut surface of the left ventricle did not show an area of myocardial infarction, and microscopic examination revealed no ischemic changes in the myocardium and subendocardium. Our patient represents the second instance of absent proximal coronary arteries with unique cardiocoronary fistulas arising from the hypoplastic right ventricle. The first case reported by Lenox and Brine9 resemblesour
Fig. 3. Fistulous connection between the right upper channel and the hypoplastic right ventricle is demonstrated. Arrow showsthe left upper channel. RV = right ventricle.
case,except that their casehad no stenosisof the tricuspid valve. In these two cases,there are common demerits concerning the coronary circulation. First, only systemic venous blood is available to the coronary circulation. Second, the coronary blood flow is not sufficiently sustained because of the direct connection between the coronary arteries and the right ventricle. Moreover, severe stenosisof the tricuspid valve in our caseobstructs inflow of blood to the right ventricle. Unfavorable coronary circulation due to these associated cardiac anomalies probably shortened survival to 4 days, in contrast with 2 months in the other reported case.’Absenceof myocardial and subendocardial ischemic changes by microscopic
598
Brief
September, 1983 American Heart Journal
Communications
Combined phenylephrine and tranylcypromine for postural hypotension Harold D. Itskovitz, M.D., and Alan Wartenburg, M.D. Milwaukee,
Fig. 4. Fistulous connection between the lower channel, which suppliesthe short apical coronary arteries, and the hypoplastic right ventricle is demonstrated. Ao = aorta; R V = right ventricle; L V = left ventricle; PA = pulmonary artery.
Fig. 5. Coronary ostium or dimple wasnot demonstrated in any aortic sinus. Ao = aorta; LV = left ventricle.
examination in the present caseis probably due to the shorter survival period. Embryogenesis of this lesion is very interesting but is by no meansclear. It is reasonable to consider the multiple fistulous communications between the right ventricle and the coronary arteries in this heart as an enlargement and persistence of the arteriosinusoidal and arterioluminal connections, which are well established before development of the coronary circulation.’ REFERENCES
1. Lenox CC, Briner J: Absent proximal coronary arteries associated with pulmonary atresia. Am J Cardiol 30~666, 1972. 2. Goor DA, Lillehei CW: Congenital malformations of the heart. New York, San Francisco, London, 1975, Grune & Stratton, Inc, p 84.
Wise.
Orthostatic hypotension is difficult to treat and many patients with this condition are severely disabled for life. We observed a female with postural hypotension (40/O mm Hg) and syncope following an acute viral infection. In addition, shedeveloped a paralytic bladder, absent sweating, and no pulsechangefollowing the Vdsalva maneuver. Her 24-hour urinary excretion of norepinephrine was minimal. Total blood volume wasnormal and there wasno laboratory evidence of abnormal pituitary, adrenal, or cardiac function to explain the patient’s postural hypotension. Our evaluation pointed to sympathetic nervous dysfunction. She was treated with intravenous saline, 9 alpha-fludrocortisone, indomethacin,’ and elastic stockings without relief of symptoms or increase in standing blood pressure(BP). Phenylephrine (4 mg/ml) by mouth to a doseof 30 mg every 4 hours gave inconsistent results and occasionally causedsupine hypertension with orthostatic hypotension. Treatment with the monoamine oxidase inhibitor (MAOI), tranylcypromine (10 mg twice daily), plus a high tyramine diet had no effect. However, when oral phenylephrine (10 to 15mg every 4-6 hours) was combined with tranylcypromine (10 mg twice daily), her supine BP reached 140 to 160/100 mm Hg and her standing systolic BP increased to 120 mm Hg. Pressor responsesoccurred 20 to 30 minutes after ingestion of phenylephrine and lasted 4 to 6 hours. With this regimen the patient’s BP wascontrolled for 6 months with few dose adjustments. Similar therapy was used successfully in three other patients. Dosesof phenylephrine ranged from 8 to 15 mg, four times daily. MAOIs have been prescribed for treatment of orthostatic hypotension with variable success2sJGenerally, these are usedwith ephedrine, tyramine, or foods high in tyramine. MAOIs benefit postural hypotension by decreasing degradation of norepinephrine and epinephrine to increasecirculating levels of endogenousvasoconstrictors. Also, they inhibit MAO in the gut and liver to allow greater gastrointestinal absorption of ingestedtyramine.4The tyramine increasesBP indirectly by enhancing the neural releaseof norepinephrine. Similarly, ephedrine releasesnorepinephrine, contributing in part to its hypertensive effect. Also, ephedrine is a beta-receptor agonist which increasesBP by increasing cardiac output. In each of these cases,vasoconstrictor effects depend upon the neural releaseof norepinephrine. However, drugs which act by releasing norepinephrine may be ineffective in patients with sympathetic lesions if norepinephrine is From
the Department
Reprint Medical
requests: College,
Harold Valhalla,
of Medicine, D. Itskovitz, NY 10595.
Medical M.D.,
College Munger
of Wisconsin. Pavilion,
New
York