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Congenital Levojuxtaposition of the Right Atrial Appendage
Congenital Levojuxtaposition of the Right Atrial Appendage * Association with Persistent Truncus Arteriosus, Type 4 Myung K. Park, M.D .; C. H. Joseph Chang, M.D .; and Thamnium Vaseenon, M.D.
Congenitallevojuxtaposition of the right atrial appendage is a rare malformation most commonly associated with a transposition of the great arteries. We report a case of levojuxtaposition of the right atrial appendage associated with a persistent truncus arteriosus, type 4. Juxtaposition of the atrial appendage is a sign of severe cyanotic congenital heart disease, and this is best diagnosed by a selective right atriogram or superior venacavogram.
J
uxtaposition of the atrial appendages refers to the condition in which both atrial appendages lie beside each other and to one side of the great vessels, a rare congenital malformation, The left-sided juxtaposition is much more frequent than the right. This condition is usually associated with complicated cardiac lesions, the most common associated defects being complete transposition of the great arteries with or without tricuspid atresia. Less commonly encountered defects are the double-outlet right ventricle and corrected transposition of the great vessels. Only three cases of levojuxtaposition with normally related great arteries have previously been reported.':" ·From the Pediatric Cardiology Section, Department of Pediatrics, and the Department of Diagnostic Radiology, University of Kansas Medical Center, Kansas City.
The purpose of this paper is to report a case of levojuxtaposition of the right atrial appendage associated with a persistent truncus arteriosus, type 4, and to review the literature pertaining to this malformation. To the best of our knowledge, persistent truncus arteriosus has not been reported previously with a levojuxtaposition of the right atrial appendage. CASE REPoRT
A six-week-old black boy was admitted to the University of Kansas Medical Center, Kansas City, for the evaluation of a heart murmur and cyanosis. The infant was born four weeks prematurely, with a birth weight of 2,750 gm (6 lb 1 oz) . The prenatal course and immediate postnatal course were uneventful. At six weeks of age, the infant was noted to have a heart murmur, tachycardia, tachypnea, and possible cyanosis. Physical examination on admission revealed a six-week-old, slightly cyanotic black infant with tachypnea (respiratory rate, 70/min), dyspnea, and mucopurulent nasal discharge. The heart rate was 160 beats per minute, and the blood pressure was 80 rom Hg by palpation, with somewhat bounding peripheral pulses. His weight on admission was 2,875 gm (6 lb 5 oz), a gain of only 125 gm (4 oz) since birth. There were bilateral rhonchi audible over the chest . The examination of the cardiovascular system revealed biventricular impulse without palpable thrill; the second heart sound was loud and single, with a prominent third heart sound at the apex. A grade 3/6, high-pitched continuous murmur was loudest over upper portion of the right side of the chest. A grade 2/6 short systolic murmur was also audible in the fourth intercostal space at the left sternal border. The liver was palpable 4 em below the right costal margin. The electrocardiogram revealed sinus tachycardia, right axis deviation (+ 150· ), and combined ventricular hypertrophy. The chest roentgenograms demonstrated moderate cardiomegaly involving both ventricles. The pulmonary vascular markings were increased, particularly on the right
FIGURE 1. Selective right arteriogram. Anteroposterior (A, left) and lateral (B, right) views showing levodorsally located right atrial appendage (raa).
550 PARK, CHANG, VASEENON
CHEST, 69: 4, APRIL, 1976
2. Right ventricular phase. Anteroposterior (A, left) and lateral (B, right) views showing right atrial appendage (raa) and persistent truncus arteriosus (TR) which is displaced cephalad, dorsally, and to left. Note agenesis of pulmonary artery and large, tortuous, and dilated bronchial arteries (arrow) supplying pulmonary circulation.
FIGURE
side, with tortuous vasculatures. The arterial blood gas analysis on admission revealed a pH of 7.26, arterial PD:! of 28 mm Hg, and arterial Pcos of 45 mm Hg. The patient received digitalis, with considerable improvement of congestive heart failure, and underwent cardiac catheterization on the following day. The salient findings included an interatrial communication with a bidirectional shunt, a ventricular septal defect, a systemic pressure in the right ventricle, a wide pulse pressure in the aorta, and moderate systemic arterial desaturation. The catheter entered the aorta from the right ventricle, but the pulmonary artery was not entered. Selective right atriogram (Fig 1 lind 2) showed a levadorsal position of the right atrial appendage. A persistent truncus arteriosus with agenesis of the pulmonary artery was also demonstrated. The pulmonary circulation was supplied by large, tortuous, and dilated bronchial arteries which arose from the proximal descending thoracic aorta. The aortic arch and the descending thoracic aorta were situated on the right. The levopositioned right atrial appendage appeared lying slightly medial and anterior to the origin of the truncus arteriosus which was displaced cephalad, dorsally, and to the left (Fig 2) . Because of the inoperability of the defects, no surgical intervention was recommended. When last seen in the outpatient clinic at the age of five months, this cyanotic infant had gained very little, with a weight of 3,300 gm (7 lb 5 oz). The infant was receiving 0.03 mg of digoxin (Lanoxin) twice daily, and the congestive heart failure was well under control. DISCUSSION
In an extensive review of 42 cases of juxtaposition of the atrial appendages, including 21 cases of their own, Melhuish and Van Praaghs noted transposition of the great arteries in over 90 percent of the cases. These included complete transposition of the great vessels with
CHEST, 69: 4, APRIL, 1976
or without tricuspid atresia, corrected transposition of the great arteries, and double-outlet right ventricle. A higher incidence of associated anomalies, such as atrial septal defect of the secundum type, ventricular septal defect, small or absent right ventricular sinus, bilateral conus, and pulmonary and aortic outflow obstruction were found more commonly in patients with juxtaposition than in patients without it, when compared with a control group of patients with transposition." Charuzi et al,3 in reviewing their 16 cases of juxtaposition, noted a similar incidence of associated cardiac anomalies. Left-sided juxtaposition was usually associated with dextrobulboventricular loop and right-sided juxtaposition with levobulboventricular loop. There were only three cases of left-sided juxtaposition which were not associated with transpositionv" and four cases of rightsided juxtaposition in the absence of transposition.s-v" Three cases of left-sided juxtaposition which were not associated with transposition had tricuspid atresia in common; these included a case of tricuspid atresia with dextrocardia and situs solitus,1,2 a case of isolated ventricular inversion with aortic and tricuspid atresia,1,2 and a case of isolated tricuspid atresia. S Among more than 70 reported cases of juxtaposition of the atrial appendage in the literature, none had persistent truncus arteriosus as an associated defect. To our knowledge, this is the first reported case of such an association. Our patient has dextroventricular loop with levocardia and situs solitus and has no evidence of tricuspid valvular abnormalities. Ellis and jameson" described an unusually flat and inconspicuous contour of the right cardiac border and two convexities along the left cardiac border as routine chest roentgenographic findings in this anomaly; however, the definite diagnosis of juxtaposition of the right
CONGENITAL LEYOJUXTAPOSmON OF RIGHT ATRIAL APPENDAGE 551
Bronchiectasis following Heroin-Induced Pulmonary Edema * Rapid Oearing of Pulmonary Infiltrates Arthur S. Banner, M.D.;oO Petham Muthuswamy, M.D.;t Ranchhodal S. Shah, M.D.;t Justo Rodriguez, M.D.;t Franklin S. Saksena, M.D.;§ and Whitney W. Addington, M.D., F.C.C.P.§
FIGURE 3. Levophase showing normally positioned left atrial appendage (LA) . Compare with Figures 1 and 2. Right atrial appendage is lying cephalad to left atrial appendage.
atrial appendage can only be made by an angiocardiogram, especially with a selective right atriogram or a superior venacavogram. Both frontal and lateral projections are essential to demonstrate the levopositioned and posteriorly located right atrial appendage. The right atrial appendage usually protrudes to the left and behind the origin of the great vessels. The right atrial appendage is also lying cephalad to the left atrial appendage (Fig 3). The right atrial appendage is usually larger than the left and shows a horn-like configuration. Angiographically, the right atrial appendage is usually not too difficult to differentiate from a left atrial appendage. REFERENCES
1 Smyth NPD : Lateroposition of the atrial appendages : A case of levoposition of the appendages. Arch Pathol60:259266, 1955 2 Melhuish BPP, Van Praagh R: Juxtaposition of the atrial appendages: A sign of severe cyanotic congenital heart disease. Br Heart J 30 :269-284, 1968 3 Charuzi Y, Spanos PK, Amplatz K, et al: Juxtaposition of the atrial appendages. Circulation 47 :620-627, 1973 4 Becker AE, Becker MJ: Juxtaposition of atrial appendages associated with normally oriented ventricles and great arteries. Circulation 41 :685-686,1970 5 Wagner HR, Adlay LE, Vlad P: Juxtaposition of the atrial appendages : A report of six necropsied cases. Circulation 42:157-163,1970 6 Deutsch V, Shem-Tov A, Yahini JH , et al : Juxtaposition of atrial appendages : Angiocardiographic observations. Am J CardioI34 :240-244, 1974 7 Ellis K, Jameson G: Congenital levoposition of the right atrial appendage. Am J RoentgenoI89:984-988, 1963
552 BANNER ET AL
We observed a patient who developed diffuse bronchiectasis subsequent to heroin-induced pulmonary edema. Unllke the previously reported cases, there was rapid clearing of pulmonary infiltrates and Utt1e evidence of severe aspiration. The development of bronchiectasis was attributed to a bronchial infection snbsequent to clearing of the pulmonary edema. Physiologic dysfunction was characterized by marked obstruction, pulmonary hypertension, and mUd hypoxemia. pulmonary impairment is considered to be R esidual minimal following heroin-induced pulmonary
edema; 1 however, there are three reported cases of lung disease due to bronchiectasis following heroin abuse.v" In each the resolution of the pulmonary infiltrates was slow, and the resulting impairment was attributed to aspiration. The present patient developed diffuse bronchiectasis following heroin-induced pulmonary edema that was characterized by a rapid clearing of the infiltrates and little evidence of severe aspiration. CASE REpORT
A 22-year-old man was admitted on Aug 8, 1975 in a stupor and with pulmonary edema following an intravenous injection of heroin. A chest roentgenogram showed diffuse inflltrates (Fig 1). The patient was intubated and treated with clindamycin and steroids, though there was little evidence of aspiration. Pneumothorax complicated a subclavian catheter insertion and was treated by insertion of a thoracostomy tube. Except for transient fever to 38°C (100.4°F) on the second hospital day, the patient remained afebrile, with minimal production of pathogen-free sputum. He was extubated on Aug 12, 1974; and on Aug 13, 1974, the chest roentgenogram was clear (Fig 2). The patient did well until Aug 20, 1974, when he began to produce large quantities of sputum which was purulent but free of pathogens. He was also noted to be profoundly hypoxemic (Table 1). Chest roentgenograms revealed bibasilar peribronchial inflltrates and a small right pleural effusion. A transient pericardial rub was heard, and an electrocardiogram revealed ST-segment elevations consistent with pericarditis. The patient was °From the Departments of Medicine and Radiology, Cook County Hospital, and Northwestern University Medical School, Chicago. 00 Instructor of Medicine. tFellow, Division of Pulmonary Medicine, Cook County Hospital. tAttending Physician, Department of Radiology, Cook County Hospital. §Assistant Professor of Medicine.
CHEST, 69: 4, APRIL, 1976
Congenitallevojuxtaposition of the right atrial appendage is a rare malformation most commonly associated with a transposition of the great arteries. We report a case of levojuxtaposition of the right atrial appendage associated with a persistent truncus arteriosus, type 4. Juxtaposition of the atrial appendage is a sign of severe cyanotic congenital heart disease, and this is best diagnosed by a selective right atriogram or superior venacavogram.
J
uxtaposition of the atrial appendages refers to the condition in which both atrial appendages lie beside each other and to one side of the great vessels, a rare congenital malformation, The left-sided juxtaposition is much more frequent than the right. This condition is usually associated with complicated cardiac lesions, the most common associated defects being complete transposition of the great arteries with or without tricuspid atresia. Less commonly encountered defects are the double-outlet right ventricle and corrected transposition of the great vessels. Only three cases of levojuxtaposition with normally related great arteries have previously been reported.':" ·From the Pediatric Cardiology Section, Department of Pediatrics, and the Department of Diagnostic Radiology, University of Kansas Medical Center, Kansas City.
The purpose of this paper is to report a case of levojuxtaposition of the right atrial appendage associated with a persistent truncus arteriosus, type 4, and to review the literature pertaining to this malformation. To the best of our knowledge, persistent truncus arteriosus has not been reported previously with a levojuxtaposition of the right atrial appendage. CASE REPoRT
A six-week-old black boy was admitted to the University of Kansas Medical Center, Kansas City, for the evaluation of a heart murmur and cyanosis. The infant was born four weeks prematurely, with a birth weight of 2,750 gm (6 lb 1 oz) . The prenatal course and immediate postnatal course were uneventful. At six weeks of age, the infant was noted to have a heart murmur, tachycardia, tachypnea, and possible cyanosis. Physical examination on admission revealed a six-week-old, slightly cyanotic black infant with tachypnea (respiratory rate, 70/min), dyspnea, and mucopurulent nasal discharge. The heart rate was 160 beats per minute, and the blood pressure was 80 rom Hg by palpation, with somewhat bounding peripheral pulses. His weight on admission was 2,875 gm (6 lb 5 oz), a gain of only 125 gm (4 oz) since birth. There were bilateral rhonchi audible over the chest . The examination of the cardiovascular system revealed biventricular impulse without palpable thrill; the second heart sound was loud and single, with a prominent third heart sound at the apex. A grade 3/6, high-pitched continuous murmur was loudest over upper portion of the right side of the chest. A grade 2/6 short systolic murmur was also audible in the fourth intercostal space at the left sternal border. The liver was palpable 4 em below the right costal margin. The electrocardiogram revealed sinus tachycardia, right axis deviation (+ 150· ), and combined ventricular hypertrophy. The chest roentgenograms demonstrated moderate cardiomegaly involving both ventricles. The pulmonary vascular markings were increased, particularly on the right
FIGURE 1. Selective right arteriogram. Anteroposterior (A, left) and lateral (B, right) views showing levodorsally located right atrial appendage (raa).
550 PARK, CHANG, VASEENON
CHEST, 69: 4, APRIL, 1976
2. Right ventricular phase. Anteroposterior (A, left) and lateral (B, right) views showing right atrial appendage (raa) and persistent truncus arteriosus (TR) which is displaced cephalad, dorsally, and to left. Note agenesis of pulmonary artery and large, tortuous, and dilated bronchial arteries (arrow) supplying pulmonary circulation.
FIGURE
side, with tortuous vasculatures. The arterial blood gas analysis on admission revealed a pH of 7.26, arterial PD:! of 28 mm Hg, and arterial Pcos of 45 mm Hg. The patient received digitalis, with considerable improvement of congestive heart failure, and underwent cardiac catheterization on the following day. The salient findings included an interatrial communication with a bidirectional shunt, a ventricular septal defect, a systemic pressure in the right ventricle, a wide pulse pressure in the aorta, and moderate systemic arterial desaturation. The catheter entered the aorta from the right ventricle, but the pulmonary artery was not entered. Selective right atriogram (Fig 1 lind 2) showed a levadorsal position of the right atrial appendage. A persistent truncus arteriosus with agenesis of the pulmonary artery was also demonstrated. The pulmonary circulation was supplied by large, tortuous, and dilated bronchial arteries which arose from the proximal descending thoracic aorta. The aortic arch and the descending thoracic aorta were situated on the right. The levopositioned right atrial appendage appeared lying slightly medial and anterior to the origin of the truncus arteriosus which was displaced cephalad, dorsally, and to the left (Fig 2) . Because of the inoperability of the defects, no surgical intervention was recommended. When last seen in the outpatient clinic at the age of five months, this cyanotic infant had gained very little, with a weight of 3,300 gm (7 lb 5 oz). The infant was receiving 0.03 mg of digoxin (Lanoxin) twice daily, and the congestive heart failure was well under control. DISCUSSION
In an extensive review of 42 cases of juxtaposition of the atrial appendages, including 21 cases of their own, Melhuish and Van Praaghs noted transposition of the great arteries in over 90 percent of the cases. These included complete transposition of the great vessels with
CHEST, 69: 4, APRIL, 1976
or without tricuspid atresia, corrected transposition of the great arteries, and double-outlet right ventricle. A higher incidence of associated anomalies, such as atrial septal defect of the secundum type, ventricular septal defect, small or absent right ventricular sinus, bilateral conus, and pulmonary and aortic outflow obstruction were found more commonly in patients with juxtaposition than in patients without it, when compared with a control group of patients with transposition." Charuzi et al,3 in reviewing their 16 cases of juxtaposition, noted a similar incidence of associated cardiac anomalies. Left-sided juxtaposition was usually associated with dextrobulboventricular loop and right-sided juxtaposition with levobulboventricular loop. There were only three cases of left-sided juxtaposition which were not associated with transpositionv" and four cases of rightsided juxtaposition in the absence of transposition.s-v" Three cases of left-sided juxtaposition which were not associated with transposition had tricuspid atresia in common; these included a case of tricuspid atresia with dextrocardia and situs solitus,1,2 a case of isolated ventricular inversion with aortic and tricuspid atresia,1,2 and a case of isolated tricuspid atresia. S Among more than 70 reported cases of juxtaposition of the atrial appendage in the literature, none had persistent truncus arteriosus as an associated defect. To our knowledge, this is the first reported case of such an association. Our patient has dextroventricular loop with levocardia and situs solitus and has no evidence of tricuspid valvular abnormalities. Ellis and jameson" described an unusually flat and inconspicuous contour of the right cardiac border and two convexities along the left cardiac border as routine chest roentgenographic findings in this anomaly; however, the definite diagnosis of juxtaposition of the right
CONGENITAL LEYOJUXTAPOSmON OF RIGHT ATRIAL APPENDAGE 551
Bronchiectasis following Heroin-Induced Pulmonary Edema * Rapid Oearing of Pulmonary Infiltrates Arthur S. Banner, M.D.;oO Petham Muthuswamy, M.D.;t Ranchhodal S. Shah, M.D.;t Justo Rodriguez, M.D.;t Franklin S. Saksena, M.D.;§ and Whitney W. Addington, M.D., F.C.C.P.§
FIGURE 3. Levophase showing normally positioned left atrial appendage (LA) . Compare with Figures 1 and 2. Right atrial appendage is lying cephalad to left atrial appendage.
atrial appendage can only be made by an angiocardiogram, especially with a selective right atriogram or a superior venacavogram. Both frontal and lateral projections are essential to demonstrate the levopositioned and posteriorly located right atrial appendage. The right atrial appendage usually protrudes to the left and behind the origin of the great vessels. The right atrial appendage is also lying cephalad to the left atrial appendage (Fig 3). The right atrial appendage is usually larger than the left and shows a horn-like configuration. Angiographically, the right atrial appendage is usually not too difficult to differentiate from a left atrial appendage. REFERENCES
1 Smyth NPD : Lateroposition of the atrial appendages : A case of levoposition of the appendages. Arch Pathol60:259266, 1955 2 Melhuish BPP, Van Praagh R: Juxtaposition of the atrial appendages: A sign of severe cyanotic congenital heart disease. Br Heart J 30 :269-284, 1968 3 Charuzi Y, Spanos PK, Amplatz K, et al: Juxtaposition of the atrial appendages. Circulation 47 :620-627, 1973 4 Becker AE, Becker MJ: Juxtaposition of atrial appendages associated with normally oriented ventricles and great arteries. Circulation 41 :685-686,1970 5 Wagner HR, Adlay LE, Vlad P: Juxtaposition of the atrial appendages : A report of six necropsied cases. Circulation 42:157-163,1970 6 Deutsch V, Shem-Tov A, Yahini JH , et al : Juxtaposition of atrial appendages : Angiocardiographic observations. Am J CardioI34 :240-244, 1974 7 Ellis K, Jameson G: Congenital levoposition of the right atrial appendage. Am J RoentgenoI89:984-988, 1963
552 BANNER ET AL
We observed a patient who developed diffuse bronchiectasis subsequent to heroin-induced pulmonary edema. Unllke the previously reported cases, there was rapid clearing of pulmonary infiltrates and Utt1e evidence of severe aspiration. The development of bronchiectasis was attributed to a bronchial infection snbsequent to clearing of the pulmonary edema. Physiologic dysfunction was characterized by marked obstruction, pulmonary hypertension, and mUd hypoxemia. pulmonary impairment is considered to be R esidual minimal following heroin-induced pulmonary
edema; 1 however, there are three reported cases of lung disease due to bronchiectasis following heroin abuse.v" In each the resolution of the pulmonary infiltrates was slow, and the resulting impairment was attributed to aspiration. The present patient developed diffuse bronchiectasis following heroin-induced pulmonary edema that was characterized by a rapid clearing of the infiltrates and little evidence of severe aspiration. CASE REpORT
A 22-year-old man was admitted on Aug 8, 1975 in a stupor and with pulmonary edema following an intravenous injection of heroin. A chest roentgenogram showed diffuse inflltrates (Fig 1). The patient was intubated and treated with clindamycin and steroids, though there was little evidence of aspiration. Pneumothorax complicated a subclavian catheter insertion and was treated by insertion of a thoracostomy tube. Except for transient fever to 38°C (100.4°F) on the second hospital day, the patient remained afebrile, with minimal production of pathogen-free sputum. He was extubated on Aug 12, 1974; and on Aug 13, 1974, the chest roentgenogram was clear (Fig 2). The patient did well until Aug 20, 1974, when he began to produce large quantities of sputum which was purulent but free of pathogens. He was also noted to be profoundly hypoxemic (Table 1). Chest roentgenograms revealed bibasilar peribronchial inflltrates and a small right pleural effusion. A transient pericardial rub was heard, and an electrocardiogram revealed ST-segment elevations consistent with pericarditis. The patient was °From the Departments of Medicine and Radiology, Cook County Hospital, and Northwestern University Medical School, Chicago. 00 Instructor of Medicine. tFellow, Division of Pulmonary Medicine, Cook County Hospital. tAttending Physician, Department of Radiology, Cook County Hospital. §Assistant Professor of Medicine.
CHEST, 69: 4, APRIL, 1976