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Extensive Multisegmental Bronchiectasis Presenting As a Transient Parahilar Coin Lesion
_
I SELECrED REPORTS
Extensive Multisegmental Bronchiectasis Presenting As a Transient Parahilar Coin Lesion" Philip M. Rogers, M.D.,oO Stephen M. Ayres, M.D.t and Charles A. Ribaudo, M.D., F.C.C.P.t
The finding of a 2 cm parahilar coin lesion in a patient with hemoptysis suggests a variety of etiologies including neoplastic disease. Of considerable interest in the present case, however, was the dlsappeanmce of the coin lesion in the upright chest roentgenogram shortly after admIssion. Tomography demonstrated cystic changes in the apex of the left lower lobe in place of the former nodular lesion. Further investigation by means of bronchography revealed the true nature of the underlying disease process, namely, saccular bronchiectasis. This report Dlustrates an unnsual radiologic presentation or bronchiectatic disease and emphasizes the need to consider the bronchiectatic sac filled with debris in the differential diagnosis of the pulmonary coin lesion.
O
ne of the intriguing aspects of bronchiectasis is its ability to simulate other entities. As the present case demonstrates, through the pursuance of radiologic investigation, well-defined bronchiectatic change may be detected in areas where, initially, it was hardly suspect. CASE REPORT
A 47-year-old black man was admitted to St. Vincent's Hospital for the first time on March 30, 1971 because of moderate hemoptysis of one day's duration. For the preceding two weeks, the patient exhibited a cough productive of yellow sputum occasionally accompanied by a dull pain in the left upper chest. The patient denied weight loss, night sweats, fever, chills or vomiting. Past medical history revealed mild essential hypertension as well as intermittent cough of six years' duration productive of whitish-yellowish sputum frequently tinged with blood. There was no history of sinusitis, tuberculosis, pneumonia or lung abscess. The patient had been smoking more than one pack of cigarettes per day since adolescence. Family history was noncontributory. His admission temperature, respiratory rate and pulse were normal; blood pressure was 150/110. The patient was well developed and of normal weight. No adenopathy, clubbing, peripheral edema, chest wall tenderness, abdominal masses °From the St. Vincent's Hospital and Medical Center and New York University Schoo of Medicinel New York City. Supported in part by USPHS National Heart Institute Training Grant No. 5-TOI-HE-05686-5, USPHS National Air Pollution Control Administration Research Grant No. ROI APOO838-01 and the Council for Tobacco Research. oOFellow in Pulmonary Diseases, Cardiopulmonary Laboratory. tDirector, Cardiopulmonary Laboratory; Associate Professor of Clinical Medicine. ;Chief, Pulmonary Diseases. Reprint requests: Dr. Ayres, 153 West 11th Street, New York City 10011
CHEST, VOL. 62, NO.4, OCTOBER, 1972
FIGURE 1. Admission PA chest film revealing a 2 x 2 em nodular density adjacent to the superior pole of the left hilum. or phlebitis were noted. The lungs were clear to percussion and auscultation and heart sounds were unremarkable. For the first two days of hospitalization the patient raised about 60 ml of predominantly bloody sputum per day, which, over the next three days, became only blood-tinged; subsequently, small amounts of yellow-white sputum were expectorated. The intermediate strength PPD skin test was positive. Of the fungal skin tests, the histoplasmin was positive. Admission WBC was 8600 with a normal differential; admission hematocrit was 40 percent. The sedimentation rate was initially 52 mm/hr (dropping to 24 within the next 48 hours). Several sputum specimens revealed only "normal throat flora." Electrocardiogram exhibited a sinus rhythm, axis +60·, nonspecific ST-T changes and poor R wave progression VI through V4. Serial SCOT, LDH, CPK and bilirubin values were normal. Admission radiologic examination revealed a smooth margined nodular lesion, approximately 2 X 2 em, immediately adjacent to the left hilum with a small area of ill-defined
FIGURE 2. Tomogram showing cystic changes in place of the former coin lesion.
499
500 infiltrate projecting above and slightly lateral to it in the PA view (Fig 1). In the same view, a dense triangular shaped calciflcation appears medially (just beneath the aortic knob) as well as a faint area of increased localized density in the right sixth rib posteriorly (probably an old bone infarct) . Lateral chest film failed to reveal any definite abnormal soft tissue mass in the hilar region; however, there was a question of minimal infiltrate in the apex of left lower lobe. The admission abdominal film exhibited neither calcified foci in the viscera nor gross organomegaly, The day after admission , AP tomograms of the "left hilar area" were obtained, the cuts beginning at the 6 em level and extending anteriorly; these films did not reveal a density that could have been considered compatible with a 2 ern solid mass lesion. Additional tomograms two days later of the more posterior regions likewise did not reveal a solid nodular lesion, but instead, one and possibly two cystic lesions with wall thickness of about 23 mm and perfectly smooth inner and outer margins (Fig 2). Therefore, it seemed reasonable to assume that the previous nodular "lesion" had been "discharged" elsewhere in the bronchial tree leaving behind an underlying cystic structure. To confirm, beyond a doubt, "disappearance" of the original nodular lesion, repeat upright chest films were taken; no longer was there any suggestion of a left suprahilar mass ( Fig 3). Furthermore, no cystic structures could be identified in these upright films. Bronchoscopy was perfonned on the ninth hospital day; concretions were not found nor were tracheal or mainstem bronchial mucosal erosions noted. Grossly, there was only minimal erythema of the left lower lobe bronchus; bronchial washings for acid-fast bacilli, fungi and malignant cells were negative; the routine culture specimen yielded normal throat Bora and very rare Proteus mirabilis. Bronchography performed on the 21st hospital day revealed, on the left, several bronchiectatic sacs, two of which were within the superior segment of the left lower lobe (Fig 4). Not well appreciated in the frontal view but seen in the oblique views were cylindrical bronchiectatic changes in the posterior segment of the left upper lobe along with a sac in this same segment. "Bronchitic" changes were not visualized. On the right side, the bronchial anatomy appeared normal,
FIGURE 3. PA chest film taken on the fifth hospital day. There is no apparent residuum of the original nodular lesion.
ROGERS, AYRES, RIBAUDO
FIGURE 4. Bronchogram, frontal view. Several bronchiectatic sacs are demonstrated on the left side. Pulmonary function evaluation done late in the patient's hospital course revealed only a mild degree of obstructive pulmonary disease, the essential abnonnality being slight reduction in Bow rate parameters. The patient signed out of the hospital and was lost to follow-up. DISCUSSION
The initial differential diagnosis included primary lung carcinoma, metastatic neoplasm to lung, bronchial adenoma, bronchogenic cyst, tuberculous or histoplasmal granuloma and pulmonary infarct. On the third hospital day, in place of the former nodular lesion, cystic structures were noted in the apex of the left lower lobe. Consequently, it became apparent that we were not dealing with a centrally located infected bronchogenic cyst.' If a granulomatous or carcinomatous lesion in the apex of the lower lobe had partially evacuated its contents, either a fluid level might have been seen in upright films or the inner wall of the lesion on tomography would probably have been irregular. Against the. diagnosis of pulmonary infarction were the absence of fever, pleural pain, effusion, or significant laboratory abnormalities, as well as the nature and rapidity of the radiologic events .t-" The original left suprahilar mass did not exhibit calcification nor was it contiguous with the densely calcified carinal lymph node; thus, it did not represent a broncholith, a solid lesion known to "disappear" occasionally without the patient being aware of expectorating a concretion" It appears, therefore, that the mass lesion represented a blood clot or mixture of blood products and pus which, in all likelihood, disintegrated and was either expectorated or spilled over elsewhere in the bronchial tree. Aside from underlying cystic bronchiectasis, one must consider two other posible etiologies for the tomographic findings: namely, emphysematous bullae which had become infected, and residual cyst formation from "healed" nonspecific lung abscess. Against the diagnosis of pan lobular emphysema was a lack of increased FRC
CHEST, VOL. 62, NO.4, OCTOBER, 1972
501
TYPE I TRUNCUS ARTERIOSUS IN ADULT and RV in pulmonary function testing. The centrilobular type of emphysema tends to affect more selectively the apices of upper and lower lobes; however, this patient did not exhibit characteristic signs or symptoms of emphysema and consequently, the existence of true emphysematous cysts (usually extremely thin walled) in this instance could be considered unlikely. The so-called post infection cyst (the "healed" residuum of nonspecific lung abscess), occasionally will develop an air-fluid level, either from a new infection to which it is susceptible or as a result of accumulation of blood within it following a hemoptysis. When the cyst is small, then sometimes called an "air space," it may become totally filled with clotted blood and consequently appear as a solid circumscribed Iesion.s However, since our patient clearly exhibited bronchiectatic changes in several bronchopulmonary segments, it is reasonable to conclude that the cyst formation in the apex of the left lower lobe was a result of bronchiectasis per se rather than intraparenchymal abscess "healing" as alluded to above. It is of interest that the pathologist can differentiate bronchiectatic cyst from post-infection (post-abscess) cyst by virtue of the fact that a small cavity, relined by epithelium (ie, the residual of a true abscess), will have multiple communications with adjacent bronchi." In conclusion, this case illustrates the necessity for, and value of detailed radiologic investigation of patients with hemoptysis and concomitant, not readily explainable pulmonary densities. The patient with a hemoptysis who exhibits a pulmonary nodular lesion must not be assumed to have bleeding secondary to a malignant neoplastic process. As demonstrated in the present case, an infected bronchiectatic cyst has the potential to fill completely with inflammatory debris before undergoing drainage, and hence, to transiently simulate a coin lesion. It becomes important, therefore, to urge that saccular bronchiectasis be included in the differential diagnosis of entities predisposing to roentgenographic pulmonary nodule formation. ACKNOWLEDGMENT: We wish to extend our appreciation to Dr. William J. Grace, Chairman, Department of Medicine, St. Vincent's Hospital and Medical Center of New York, for carefully reviewing the manuscript. We are also grateful to all the members of the Department of Radiology who cooperated so fully in studying the patient. REFERENCES
1 Golden's Diagnostic Roentgenology, Vol 2. Robbins LL, Ed. Baltimore, Williams & Wilkins, 1967, 185Q 2 Laforet EG, Laforet MT: Nontuberculous cavitary disease of the lungs. Dis Chest 31:665, 1957 3 Parker BM, Smith JR: Pulmonary emoblism and infarction: A review of the physiologic consequences of pulmonary arterial obstruction. Am J Med 24:402, 1958 4 Rosenow EC, Fontana FR, Andersen HA: Spontaneous disappearance of a calcified solitary pulmonary nodule. Chest 59:338-340, 1971 5 Golden's Diagnostic Roentgenology, Vol 2. Robbins LL, Ed. Baltimore, Williams & Wilkins, 1967, 188H-I881 6 Lindskog GE, Liebow AA, Glenn WWL: Thoracic and Cardiovascular Surgery with Related Pathology. New York, Appleton-Century-Crofts, 1962,162
CHEST, VOL. 62, NO.4, OCTOBER, 1972
Type I Truncus Arteriosus Adult*
In
an
Jacob R. Morgan, CAPT, MC, USN, F.C.C.P.; Donald J. Kearney, LCDR, MC, USN and Arthur D. Hagan, CDR, MC, USN, F.C.C.P.
A 24-year-old woman is the oldest patient in whom a diagnosis of true truncus arteriosus was made during life and confirmed by cineangiography. The patient has also had the longest known survival with a type I truncus arteriosus. Clinical findings were of interest. he diagnosis of true persistent truncus arteriosus T during life in an adult has not been reported previ-
ously. The patient reported here has had the longest reported survival with a type I truncus arteriosus. The clinical findings of a long, harsh systolic murmur and the chest roentgenogram in this cyanotic patient were suggestive of the diagnosis. Of further interest was the "slightly split" second heart sound. CASE REPoRT
This 24-year-old white woman has been cyanotic since birth. She had pneumonia three times as a young child. Strenuous exertion during childhood resulted in syncope. Cardiac catheterization at another institution, in 1958, was stated to show a ventricular septal defect with a bidirectional shunt. Two months after being placed on birth control pills, in 1969, she had thrombophlebitis in her left leg. Three grandmal seizures in 1970 were considered due to cerebral thrombosis. At the present time she is active and has shortness of breath only after climbing two flights of stairs. Physical Examination
The patient was 58 inches tall, weighed 101 pounds and had moderate cyanosis and clubbing. Blood pressure was 130170. A prominent A wave was visible in the jugular pulse. There was a right ventricular lift and along the left sternal border, a prominent systolic ejection click. The second heart sound was loud and by auscultation narrowly split (not a pure single sound). The splitting appeared to be due to vibrations of the "aortic" second sound on the phonocardiogram (Fig 1). A harsh grade 4/6 holosystolic murmur was heard best at the fourth left intercostal space. There was also a short grade 1/6 diastolic decrescendo murmur along the left sternal border. Hemoglobin was 18 gm percent and hematocrit 56 percent. On chest roentgenogram (Fig 2) the right and left pulmonary arteries were higher than usual and there was right ventricular enlargement. Electrocardiogram and vectorcardiogram showed right axis deviation and right ventricular hypertrophy. Catheterization and Cineangiography
The systolic pressure in the right ventricle, pulmonary ·From the Department of Cardiology and the Clinical Investigation Center, U.S. Naval Hospital, San Diego, California. The opinions or assertions contained herein are those of the authors and are not to be construed as official or as reflecting the views of the Department of the Navy. Reprint requests: Capt. Morgan, Naval Hospital, San Diego
92134
I SELECrED REPORTS
Extensive Multisegmental Bronchiectasis Presenting As a Transient Parahilar Coin Lesion" Philip M. Rogers, M.D.,oO Stephen M. Ayres, M.D.t and Charles A. Ribaudo, M.D., F.C.C.P.t
The finding of a 2 cm parahilar coin lesion in a patient with hemoptysis suggests a variety of etiologies including neoplastic disease. Of considerable interest in the present case, however, was the dlsappeanmce of the coin lesion in the upright chest roentgenogram shortly after admIssion. Tomography demonstrated cystic changes in the apex of the left lower lobe in place of the former nodular lesion. Further investigation by means of bronchography revealed the true nature of the underlying disease process, namely, saccular bronchiectasis. This report Dlustrates an unnsual radiologic presentation or bronchiectatic disease and emphasizes the need to consider the bronchiectatic sac filled with debris in the differential diagnosis of the pulmonary coin lesion.
O
ne of the intriguing aspects of bronchiectasis is its ability to simulate other entities. As the present case demonstrates, through the pursuance of radiologic investigation, well-defined bronchiectatic change may be detected in areas where, initially, it was hardly suspect. CASE REPORT
A 47-year-old black man was admitted to St. Vincent's Hospital for the first time on March 30, 1971 because of moderate hemoptysis of one day's duration. For the preceding two weeks, the patient exhibited a cough productive of yellow sputum occasionally accompanied by a dull pain in the left upper chest. The patient denied weight loss, night sweats, fever, chills or vomiting. Past medical history revealed mild essential hypertension as well as intermittent cough of six years' duration productive of whitish-yellowish sputum frequently tinged with blood. There was no history of sinusitis, tuberculosis, pneumonia or lung abscess. The patient had been smoking more than one pack of cigarettes per day since adolescence. Family history was noncontributory. His admission temperature, respiratory rate and pulse were normal; blood pressure was 150/110. The patient was well developed and of normal weight. No adenopathy, clubbing, peripheral edema, chest wall tenderness, abdominal masses °From the St. Vincent's Hospital and Medical Center and New York University Schoo of Medicinel New York City. Supported in part by USPHS National Heart Institute Training Grant No. 5-TOI-HE-05686-5, USPHS National Air Pollution Control Administration Research Grant No. ROI APOO838-01 and the Council for Tobacco Research. oOFellow in Pulmonary Diseases, Cardiopulmonary Laboratory. tDirector, Cardiopulmonary Laboratory; Associate Professor of Clinical Medicine. ;Chief, Pulmonary Diseases. Reprint requests: Dr. Ayres, 153 West 11th Street, New York City 10011
CHEST, VOL. 62, NO.4, OCTOBER, 1972
FIGURE 1. Admission PA chest film revealing a 2 x 2 em nodular density adjacent to the superior pole of the left hilum. or phlebitis were noted. The lungs were clear to percussion and auscultation and heart sounds were unremarkable. For the first two days of hospitalization the patient raised about 60 ml of predominantly bloody sputum per day, which, over the next three days, became only blood-tinged; subsequently, small amounts of yellow-white sputum were expectorated. The intermediate strength PPD skin test was positive. Of the fungal skin tests, the histoplasmin was positive. Admission WBC was 8600 with a normal differential; admission hematocrit was 40 percent. The sedimentation rate was initially 52 mm/hr (dropping to 24 within the next 48 hours). Several sputum specimens revealed only "normal throat flora." Electrocardiogram exhibited a sinus rhythm, axis +60·, nonspecific ST-T changes and poor R wave progression VI through V4. Serial SCOT, LDH, CPK and bilirubin values were normal. Admission radiologic examination revealed a smooth margined nodular lesion, approximately 2 X 2 em, immediately adjacent to the left hilum with a small area of ill-defined
FIGURE 2. Tomogram showing cystic changes in place of the former coin lesion.
499
500 infiltrate projecting above and slightly lateral to it in the PA view (Fig 1). In the same view, a dense triangular shaped calciflcation appears medially (just beneath the aortic knob) as well as a faint area of increased localized density in the right sixth rib posteriorly (probably an old bone infarct) . Lateral chest film failed to reveal any definite abnormal soft tissue mass in the hilar region; however, there was a question of minimal infiltrate in the apex of left lower lobe. The admission abdominal film exhibited neither calcified foci in the viscera nor gross organomegaly, The day after admission , AP tomograms of the "left hilar area" were obtained, the cuts beginning at the 6 em level and extending anteriorly; these films did not reveal a density that could have been considered compatible with a 2 ern solid mass lesion. Additional tomograms two days later of the more posterior regions likewise did not reveal a solid nodular lesion, but instead, one and possibly two cystic lesions with wall thickness of about 23 mm and perfectly smooth inner and outer margins (Fig 2). Therefore, it seemed reasonable to assume that the previous nodular "lesion" had been "discharged" elsewhere in the bronchial tree leaving behind an underlying cystic structure. To confirm, beyond a doubt, "disappearance" of the original nodular lesion, repeat upright chest films were taken; no longer was there any suggestion of a left suprahilar mass ( Fig 3). Furthermore, no cystic structures could be identified in these upright films. Bronchoscopy was perfonned on the ninth hospital day; concretions were not found nor were tracheal or mainstem bronchial mucosal erosions noted. Grossly, there was only minimal erythema of the left lower lobe bronchus; bronchial washings for acid-fast bacilli, fungi and malignant cells were negative; the routine culture specimen yielded normal throat Bora and very rare Proteus mirabilis. Bronchography performed on the 21st hospital day revealed, on the left, several bronchiectatic sacs, two of which were within the superior segment of the left lower lobe (Fig 4). Not well appreciated in the frontal view but seen in the oblique views were cylindrical bronchiectatic changes in the posterior segment of the left upper lobe along with a sac in this same segment. "Bronchitic" changes were not visualized. On the right side, the bronchial anatomy appeared normal,
FIGURE 3. PA chest film taken on the fifth hospital day. There is no apparent residuum of the original nodular lesion.
ROGERS, AYRES, RIBAUDO
FIGURE 4. Bronchogram, frontal view. Several bronchiectatic sacs are demonstrated on the left side. Pulmonary function evaluation done late in the patient's hospital course revealed only a mild degree of obstructive pulmonary disease, the essential abnonnality being slight reduction in Bow rate parameters. The patient signed out of the hospital and was lost to follow-up. DISCUSSION
The initial differential diagnosis included primary lung carcinoma, metastatic neoplasm to lung, bronchial adenoma, bronchogenic cyst, tuberculous or histoplasmal granuloma and pulmonary infarct. On the third hospital day, in place of the former nodular lesion, cystic structures were noted in the apex of the left lower lobe. Consequently, it became apparent that we were not dealing with a centrally located infected bronchogenic cyst.' If a granulomatous or carcinomatous lesion in the apex of the lower lobe had partially evacuated its contents, either a fluid level might have been seen in upright films or the inner wall of the lesion on tomography would probably have been irregular. Against the. diagnosis of pulmonary infarction were the absence of fever, pleural pain, effusion, or significant laboratory abnormalities, as well as the nature and rapidity of the radiologic events .t-" The original left suprahilar mass did not exhibit calcification nor was it contiguous with the densely calcified carinal lymph node; thus, it did not represent a broncholith, a solid lesion known to "disappear" occasionally without the patient being aware of expectorating a concretion" It appears, therefore, that the mass lesion represented a blood clot or mixture of blood products and pus which, in all likelihood, disintegrated and was either expectorated or spilled over elsewhere in the bronchial tree. Aside from underlying cystic bronchiectasis, one must consider two other posible etiologies for the tomographic findings: namely, emphysematous bullae which had become infected, and residual cyst formation from "healed" nonspecific lung abscess. Against the diagnosis of pan lobular emphysema was a lack of increased FRC
CHEST, VOL. 62, NO.4, OCTOBER, 1972
501
TYPE I TRUNCUS ARTERIOSUS IN ADULT and RV in pulmonary function testing. The centrilobular type of emphysema tends to affect more selectively the apices of upper and lower lobes; however, this patient did not exhibit characteristic signs or symptoms of emphysema and consequently, the existence of true emphysematous cysts (usually extremely thin walled) in this instance could be considered unlikely. The so-called post infection cyst (the "healed" residuum of nonspecific lung abscess), occasionally will develop an air-fluid level, either from a new infection to which it is susceptible or as a result of accumulation of blood within it following a hemoptysis. When the cyst is small, then sometimes called an "air space," it may become totally filled with clotted blood and consequently appear as a solid circumscribed Iesion.s However, since our patient clearly exhibited bronchiectatic changes in several bronchopulmonary segments, it is reasonable to conclude that the cyst formation in the apex of the left lower lobe was a result of bronchiectasis per se rather than intraparenchymal abscess "healing" as alluded to above. It is of interest that the pathologist can differentiate bronchiectatic cyst from post-infection (post-abscess) cyst by virtue of the fact that a small cavity, relined by epithelium (ie, the residual of a true abscess), will have multiple communications with adjacent bronchi." In conclusion, this case illustrates the necessity for, and value of detailed radiologic investigation of patients with hemoptysis and concomitant, not readily explainable pulmonary densities. The patient with a hemoptysis who exhibits a pulmonary nodular lesion must not be assumed to have bleeding secondary to a malignant neoplastic process. As demonstrated in the present case, an infected bronchiectatic cyst has the potential to fill completely with inflammatory debris before undergoing drainage, and hence, to transiently simulate a coin lesion. It becomes important, therefore, to urge that saccular bronchiectasis be included in the differential diagnosis of entities predisposing to roentgenographic pulmonary nodule formation. ACKNOWLEDGMENT: We wish to extend our appreciation to Dr. William J. Grace, Chairman, Department of Medicine, St. Vincent's Hospital and Medical Center of New York, for carefully reviewing the manuscript. We are also grateful to all the members of the Department of Radiology who cooperated so fully in studying the patient. REFERENCES
1 Golden's Diagnostic Roentgenology, Vol 2. Robbins LL, Ed. Baltimore, Williams & Wilkins, 1967, 185Q 2 Laforet EG, Laforet MT: Nontuberculous cavitary disease of the lungs. Dis Chest 31:665, 1957 3 Parker BM, Smith JR: Pulmonary emoblism and infarction: A review of the physiologic consequences of pulmonary arterial obstruction. Am J Med 24:402, 1958 4 Rosenow EC, Fontana FR, Andersen HA: Spontaneous disappearance of a calcified solitary pulmonary nodule. Chest 59:338-340, 1971 5 Golden's Diagnostic Roentgenology, Vol 2. Robbins LL, Ed. Baltimore, Williams & Wilkins, 1967, 188H-I881 6 Lindskog GE, Liebow AA, Glenn WWL: Thoracic and Cardiovascular Surgery with Related Pathology. New York, Appleton-Century-Crofts, 1962,162
CHEST, VOL. 62, NO.4, OCTOBER, 1972
Type I Truncus Arteriosus Adult*
In
an
Jacob R. Morgan, CAPT, MC, USN, F.C.C.P.; Donald J. Kearney, LCDR, MC, USN and Arthur D. Hagan, CDR, MC, USN, F.C.C.P.
A 24-year-old woman is the oldest patient in whom a diagnosis of true truncus arteriosus was made during life and confirmed by cineangiography. The patient has also had the longest known survival with a type I truncus arteriosus. Clinical findings were of interest. he diagnosis of true persistent truncus arteriosus T during life in an adult has not been reported previ-
ously. The patient reported here has had the longest reported survival with a type I truncus arteriosus. The clinical findings of a long, harsh systolic murmur and the chest roentgenogram in this cyanotic patient were suggestive of the diagnosis. Of further interest was the "slightly split" second heart sound. CASE REPoRT
This 24-year-old white woman has been cyanotic since birth. She had pneumonia three times as a young child. Strenuous exertion during childhood resulted in syncope. Cardiac catheterization at another institution, in 1958, was stated to show a ventricular septal defect with a bidirectional shunt. Two months after being placed on birth control pills, in 1969, she had thrombophlebitis in her left leg. Three grandmal seizures in 1970 were considered due to cerebral thrombosis. At the present time she is active and has shortness of breath only after climbing two flights of stairs. Physical Examination
The patient was 58 inches tall, weighed 101 pounds and had moderate cyanosis and clubbing. Blood pressure was 130170. A prominent A wave was visible in the jugular pulse. There was a right ventricular lift and along the left sternal border, a prominent systolic ejection click. The second heart sound was loud and by auscultation narrowly split (not a pure single sound). The splitting appeared to be due to vibrations of the "aortic" second sound on the phonocardiogram (Fig 1). A harsh grade 4/6 holosystolic murmur was heard best at the fourth left intercostal space. There was also a short grade 1/6 diastolic decrescendo murmur along the left sternal border. Hemoglobin was 18 gm percent and hematocrit 56 percent. On chest roentgenogram (Fig 2) the right and left pulmonary arteries were higher than usual and there was right ventricular enlargement. Electrocardiogram and vectorcardiogram showed right axis deviation and right ventricular hypertrophy. Catheterization and Cineangiography
The systolic pressure in the right ventricle, pulmonary ·From the Department of Cardiology and the Clinical Investigation Center, U.S. Naval Hospital, San Diego, California. The opinions or assertions contained herein are those of the authors and are not to be construed as official or as reflecting the views of the Department of the Navy. Reprint requests: Capt. Morgan, Naval Hospital, San Diego
92134