- Email: [email protected]
Primary Alveolar Hypoventilation Managed by Negative-Pressure Ventilators
SeL6CT6D RePORTS
CASE
A 14-year-old girl was transferred to the University of Alberta Hospital, Edmonton, for investigation of a long history of repeated spells of apnea. In infancy and early childhood, her parents first noticed frequent apnea, nocturnal snoring, and cyanosis. She was lethargic and often blue IN the morning and awakened with difficulty. There was no apparent daytime somnolence. Although the patient was scheduled for a tonsillectomy at the age offiveyears, the operation was cancelled because of cardiomegaly, basal crepitations, and radiologic evidence of pulmonary congestion. She then had several admissions to another hospital for treatment of "congestive heart failure." On two occasions, "cardiac arrest" occurred IN the hospital IN conjunction with the administration of sedative medications. A cardiac catheterization done when the patient was nine years old showed normal pressures in all chambers and no intracardiac shunting. Chest x-ray films taken at intervals were entirely normal. During a recent admission precipitated by an infection of the upper respiratory tract, the patient suffered a cardiorespiratory arrest and required intubation and therapy with mechanical ventilation. Attempts to wean her from the respirator were initially unsuccessful, as she retained carbon dioxide whenever there was no ventilatory support; however, once the resistive load of the endotracheal tube was removed, the patient was able to breathe reasonably well while awake. Physical examination showed the patient to be obese, with a body weight of 65.1 kg (143 lb) (90th percentile for her age) and a height of 160 cm (5 ft 3 in) (50th percentile). The blood pressure was 120/80 mm Hg; the pulse rate was 90 beats per minute. Examination of the chest, cardiovascular system, and central nervous system revealed no abnormality. The level of hemoglobin was 14.5 gm/100 ml. The results of an automated sequential multiple analysis of the blood (SMA-12), an electrocardiogram, a chest x-rayfilm,and tests of thyroid and adrenal function were normal. A computerized axial tomographic scan and pneumoencephalogram did not show any intracranial lesion. On neuropsychologic assessment, the patient obtained a verbal inteDigence quotient in the average range but was slightly below average in abstract reasoning. Studies of pulmonary function showed a mild restrictive defect, with no evidence of obstruction of the airways. Serial samples of arterial blood showed that the patient hypoventilated when asleep, with arterial carbon dioxide tension (PaC0 ) ranging from 70 to 90 mm Hg. When aroused, the PaC0 was maintained between 30 and 45 mm Hg. The patient had no ventilatory response to hypoxia (Fig 1) or hypercapnia (Fig 2) when tested ' while fully awake.
Primary Alveolar Hypoventilation Managed by Negative-Pressure Ventilators* Godfrey C. W. Man, M.B.;»» Richard L. Jones,
Ph.D.,
F.C.C.P.;t Giles F. MacDonald, M.D., F.C.CP.;t and
E. Garner King, M.D., F.C.C.P.% A girl who had a history of repeated apnea was found to have absent chemical drive to ventilation and, on sleep monitoring, both central and obstructive types of apnea. She is currently undergoing successful mechanical ventilation at night with negative-pressure ventilators.
"Drimary alveolar hypoventilation has been described * as a syndrome in which automatic and chemical control of ventilation is absent without associated cardiac, pulmonary, or neuromuscular disease. Recently, a syndrome of sleep-related apnea has been reported, and in general, there appear to be three types of apnea: (1) central apnea due to a primary cessation of respiratory effort; (2) apnea due to obstruction of the upper airway; and (3) mixed apnea. Considerable recent interest in the syndromes of apnea " prompts us to report the study of a patient who lacked a chemical ventilatory drive, had sleep-related apnea, and also had evidence for obstruction of the upper airway. This patient has been successfully managed at night in negative-phase tank and cuirass-type respirators. 1
2
3
REPORT
6
"From the Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Alberta, Edmonton. * "Resident in Medicine, University of Alberta Hospital, Edmonton. fAssistant Professor of Medicine. {Clinical Head, Department of Medicine, Edmonton General Hospital. §Associate Professor of Medicine and Director, Intensive Care Unit.
2
2
Reprint requests: Dr. King, 6-106 Clinical Sciences Building, University of Alberta, Edmonton
7 8
3 0
% (L/min)
2 5 20 1510-
1
3 0
3 9
4 8
57 •
• CHEST, 76: 2, AUGUST, 1979
(
66
1
75
PA02
(mmHg)
Pa02
(mmHg)
8 4
9 3
102
111
F I G U R E 1. Effects of lowering alveolar oxygen pressure ( P A O ) on ventilation ( V E ) (hypoxic response). Four samples of arterial blood were taken toward end of rebreathing period, and these show hypoxemia. Pa0 , Arterial oxygen pressure. z
—i—
- 1 —
120
2
PRIMARY ALVEOLAR HYPOVENTILATION
219
20
VE
(L/min)
system, such as encephalitis, meningitis, and syringomyelia, was present in some patients. In our patient, extensive neurologic investigation did not reveal any evidence of disease of the central nervous system. Therapy with several drugs, such as dextroamphetamine, aspirin, acetazolamide, progesterone, and theophylline, has been tried in the past in attempts to stimulate chemoreceptor ventilatory drive in such patients, all without significant effect. A thiamine inhibitor was found in two patients with primary alveolar hypoventilation, and administration of thiamine was effective in abolishing the episodes of apnea in one patient. Our patient did not show any response to therapy with progesterone, imipramine, or thiamine. Patients with the syndrome of sleep-related apnea associated with obstruction of the upper airway may have symptoms of snoring, abnormal motor activity during sleep, excessive daytime sleepiness, changes in personality, deteriorating intellectual capacity, hypertension, sexual problems, abnormal outbursts, hypnagogic hallucinations and automatic behavior, and nocturnal enuresis. Various causes for the obstruction have been implicated, ie, a thick short neck, large jowl, hypertrophic palate, and enlarged adenoids or tonsils. Recent evidence suggests that the specific site of obstruction of the airway is in the oral pharynx at the velopharyngeal sphincter. In our patient, clinical examination did not reveal any obvious cause of obstruction; however, after a tonsillectomy and adenoidectomy, the episodes of obstructive apnea could no longer be demonstrated. Hemodynamic studies done in patients with the syndrome of sleep-related apnea have shown cyclic elevations of systemic as well as pulmonary arterial pressures coinciding with the episodes of apnea. Hypoxemia and acidosis were thought to be the important causative factors. These patients may eventually develop sustained pulmonary hypertension and cor pulmonale. Tracheostomy has been found to be effective in abolishing the obstructive type of apnea, although the central apnea may persist. The coexistence of primary alveolar hypoventilation and sleep-associated obstruction of the upper airway was reported recently, and these patients were managed with electrophrenic pacing and tracheostomy. Although experience with electrophrenic pacing is accumulating, success is not universal and depends on careful selection of patients, meticulous technique in implanting the electrodes, and proper use of the apparatus. Tracheostomy 9
10
10
10
11
11
V.
— i —
46
— I —
54
50
58
1—
r—i—
62
66
70
(mmHg) PC0 F I G U R E 2. Effects of increasing carbon dioxide pressure on ventilation ( V E ) (hypercapnic response). Pco , End-tidal carbon dioxide pressure. 2
2
The father, mother, and sister had normal hypoxic and hypercapnic responses. Sleep monitoring was performed, with simultaneous recordings of ear oximetric data, arterial blood gas levels, electroencephalogram, esophageal balloon pressure, and airflowat the nose (monitored by thermistor). These studies documented multiple episodes of apnea throughout the night, some lasting over 90 seconds. Both central and obstructive types of apnea were identified (Fig 3). Recordings of the EEG showed that the patient's pattern of sleep was abnormal, with frequent arousal and predominating light stages of sleep. Rapid-eye-movement sleep was not observed over two nights. Therapy with various medications was tried, including medroxyprogesterone 17-acetate (Provera), imipramine, and thiamine, without any effect Although clinically there was no marked tonsillar enlargement, tonsillectomy and adenoidectomy were performed. On repetition of sleep monitoring, the episodes of obstructive apnea were no longer demonstrable, while episodes of central apnea persisted. At present the patient is managing very well at home, being mechanically ventilated at night with a tank respirator (Emerson). During the day, she functions well without ventilatory assistance, is able to attend school, and has no respiratory difficulties. When travelling, the patient sleeps in a cuirass respirator. DISCUSSION
9
SPONTANEOUS RESPIRATION
11
13
14
15
Severinghaus and Mitchell first used the term, "Ondine's curse," in 1972 in a report describing three patients with loss of automatic control of respiration due to surgical damage to the upper cervical cord; however, the term, "primary alveolar hypoventilation," is now preferred. Mellins et al reviewed 30 reported cases in 1970. The cause of the absent chemical drive was largely obscure, but a history of a disease of the central nervous 1
12
CENTRAL APNOEA
8
16
OBSTRUCTIVE APNOEA
Nasal Thermistor
Esophageal Balloon F I G U R E 3. Representative tracings of nasal temperature (index of air flow) and esophageal pressure obtained during four-hour period of sleep. Tracings show three types of breathing, ie, spontaneous respiration, central apnea, and obstructive apnea.
220
MAN ET AL
CHEST, 7 6 : 2,
AUGUST,
1979
appears to be often necessary, as accentuation of obstruction of the upper airway may occur with pacing. We are not aware of other patients with primary alveolar hypoventilation who are being maintained with the aid of negative-pressure respirators. Although somewhat physically unwieldy, their operation is simple; and intubation, tracheostomy, or other surgical procedures are not required. Moreover, these devices have allowed our patient to have a comfortable sleep at night and complete freedom during the day. Our experience would suggest that negative-pressure ventilators represent a reasonable conservative alternative to electrophrenic pacing for some patients with primary alveolar hypoventilation. ACKNOWLEDGMENT: We wish to thank the respiratory technicians and nursing staff of the Intensive Care Unit, University of Alberta Hospital, Mr. Allan Wells, Ms. Rhoda Cardwell, and Mrs. Margaret Napier for their kind technical assistance and Dr. John Weil for his valuable advice and comments. REFERENCES
1 Severinghaus JW, Mitchell R: Ondine's curse: Failure of respiratory center automaticity while awake. Clin Res 10:122, 1962 2 Guilleminault C, Tilkian A, Dement WC: The sleep apnea syndromes. Ann Rev Med 27:463-484, 1976 3 Whitcomb ME, Altman M, Clark RW, et al: Central and obstructive sleep apnea: Pulmonary disease conference at the Ohio State University, Columbus. Chest 73:857-860, 1978 4 Strohl KP, Saunders NA, Feldman NT, et al: Obstructive sleep apnea in family members. N Engl J Med 299:969973, 1978 5 Phillipson EA: Control of breathing during sleep. Am Rev Respir Dis 118:909-939, 1978 6 Glenn WWL, Gee JBL, Cole DR, et al: Combined central alveolar hypoventilation and upper airway obstruction. Am J Med 64:50-60,1978 7 Rebuck AS, Campbell EJM: A clinical method for assessing the ventilatory response to hypoxia. Am Rev Respir Dis 109:345-350, 1974 8 Read DJC: A clinical method for assessing the ventilatory response to carbon dioxide. Aust Ann Med 16:20-32, 1967 9 Mellins RB, Balfour HH, Turino GM, et al: Failure of automatic control of ventilation (Ondine's curse). Medicine 49:487-504, 1970 10 Deonna T, Arzynska W, Torrado A: Congenital failure of automatic ventilation (Ondine's curse). J Pediatr 84:710714, 1974 11 Guilleminault C, Eldridge FL, Tilkian A: Sleep apnea syndrome due to upper airway obstruction. Arch Intern Med 137:296-300,1977 12 Lonsdale D, Mercer RD: Primary hypoventilation syndrome. Lancet 2:487, 1972 13 Weitzman D, Pollack CP, Borowiecki B: The hypersomnia sleep apnea syndrome: Site and mechanism of upper airways obstruction. In Guilleminault C, Dement WC (eds): Sleep Apnea Syndromes. New York, Alan R Liss, Inc, 1978, pp 235-248 14 Tilkian AG, Guilleminault C, Schroeder JS: Hemodynamics in sleep-induced apnea. Ann Intern Med 85:714719, 1976 15 Sackner MA, Landa J, Forrest T, et al: Periodic sleep CHEST, 7 6 : 2 , AUGUST, 1 9 7 9
apnea: Chronic sleep deprivation related to intermittent upper airway obstruction and central nervous system disturbance. Chest 67:164-171,1975 16 Glenn WWL: Diaphragm pacing: Present status. PACE 1:357-370,1978
Angiosarcoma of the Heart* Diagnosis by Echocardiography Joseph
S. Panella,
Victor,
M.D.;
M.D.;
Ronald
David C. Hueter,
A.
Milton L. Paige, Semerdjian,
M.D.;
M.D.,
Thomas
F.C.C.P.;
A. and
M.D.
Angiosarcoma of the heart is a rare tumor infrequently diagnosed ante mortem. The clinical presentation is usually that of pericarditis or congestive heart failure. This case report illustrates the contribution of echocardiography in suggesting the diagnosis.
A
ngiosarcoma of the heart is a rare disease which is infrequently diagnosed before autopsy. Radiographic examination is occasionally helpful in suggesting the presence of the malignant neoplasm. Angiograms, pneurnopericardiograms, and routine chest x-ray films have all been reported as useful. " The following case report describes a primary cardiac angiosarcoma in which the chest x-ray film and echocardiogram suggested the presence of a cardiac tumor, which was subsequently confirmed by thoracotomy and autopsy. 1
3
CASE REPORT
A 40-year-old white man had been in good health until July 1977, when he developed sharp substernal pain. Both the chest x-ray film, which showed cardiomegaly, and the electrocardiogram were consistent with the clinical impression of pericarditis. An echocardiogram confirmed the presence of a pericardial effusion. The patient's condition initially responded to therapy with aspirin, but symptoms soon recurred. Upon readmission, an echocardiogram (Fig 1A) demonstrated the persistence of a moderate to large pericardial effusion. The patient's condition again responded to conservative management, but complaints of persistent fatigue, abdominal distention, and tightness in the chest resulted in readmission in December 1977. The chest x-rayfilmrevealed persistent cardiomegaly and diffuse nodular infiltrates. An echocardiogram (Fig IB) no longer showed a typical pericardial effusion, but rather a large echo-free space confined anterior to the free right ventricular wall. In addition, there was now marked thickening of the right ventricular epicardial surface suggestive of a clot or tumor. Because of the atypical clinical course and the unusual radiographic and echocardiographic findings, a diagnostic thoracotomy was performed. At surgery a vascular friable tumor filled the pericardial 'From the Departments of Radiology, Pathology, and Internal Medicine, Evanston Hospital and Northwestern University, Evanston, 111.
Reprint requests: Dr. Paige, Department ton Hospital, Evanston, Illinois 60201
of Radiology,
Evans-
ANGIOSARCOMA OF THE HEART 221
CASE
A 14-year-old girl was transferred to the University of Alberta Hospital, Edmonton, for investigation of a long history of repeated spells of apnea. In infancy and early childhood, her parents first noticed frequent apnea, nocturnal snoring, and cyanosis. She was lethargic and often blue IN the morning and awakened with difficulty. There was no apparent daytime somnolence. Although the patient was scheduled for a tonsillectomy at the age offiveyears, the operation was cancelled because of cardiomegaly, basal crepitations, and radiologic evidence of pulmonary congestion. She then had several admissions to another hospital for treatment of "congestive heart failure." On two occasions, "cardiac arrest" occurred IN the hospital IN conjunction with the administration of sedative medications. A cardiac catheterization done when the patient was nine years old showed normal pressures in all chambers and no intracardiac shunting. Chest x-ray films taken at intervals were entirely normal. During a recent admission precipitated by an infection of the upper respiratory tract, the patient suffered a cardiorespiratory arrest and required intubation and therapy with mechanical ventilation. Attempts to wean her from the respirator were initially unsuccessful, as she retained carbon dioxide whenever there was no ventilatory support; however, once the resistive load of the endotracheal tube was removed, the patient was able to breathe reasonably well while awake. Physical examination showed the patient to be obese, with a body weight of 65.1 kg (143 lb) (90th percentile for her age) and a height of 160 cm (5 ft 3 in) (50th percentile). The blood pressure was 120/80 mm Hg; the pulse rate was 90 beats per minute. Examination of the chest, cardiovascular system, and central nervous system revealed no abnormality. The level of hemoglobin was 14.5 gm/100 ml. The results of an automated sequential multiple analysis of the blood (SMA-12), an electrocardiogram, a chest x-rayfilm,and tests of thyroid and adrenal function were normal. A computerized axial tomographic scan and pneumoencephalogram did not show any intracranial lesion. On neuropsychologic assessment, the patient obtained a verbal inteDigence quotient in the average range but was slightly below average in abstract reasoning. Studies of pulmonary function showed a mild restrictive defect, with no evidence of obstruction of the airways. Serial samples of arterial blood showed that the patient hypoventilated when asleep, with arterial carbon dioxide tension (PaC0 ) ranging from 70 to 90 mm Hg. When aroused, the PaC0 was maintained between 30 and 45 mm Hg. The patient had no ventilatory response to hypoxia (Fig 1) or hypercapnia (Fig 2) when tested ' while fully awake.
Primary Alveolar Hypoventilation Managed by Negative-Pressure Ventilators* Godfrey C. W. Man, M.B.;»» Richard L. Jones,
Ph.D.,
F.C.C.P.;t Giles F. MacDonald, M.D., F.C.CP.;t and
E. Garner King, M.D., F.C.C.P.% A girl who had a history of repeated apnea was found to have absent chemical drive to ventilation and, on sleep monitoring, both central and obstructive types of apnea. She is currently undergoing successful mechanical ventilation at night with negative-pressure ventilators.
"Drimary alveolar hypoventilation has been described * as a syndrome in which automatic and chemical control of ventilation is absent without associated cardiac, pulmonary, or neuromuscular disease. Recently, a syndrome of sleep-related apnea has been reported, and in general, there appear to be three types of apnea: (1) central apnea due to a primary cessation of respiratory effort; (2) apnea due to obstruction of the upper airway; and (3) mixed apnea. Considerable recent interest in the syndromes of apnea " prompts us to report the study of a patient who lacked a chemical ventilatory drive, had sleep-related apnea, and also had evidence for obstruction of the upper airway. This patient has been successfully managed at night in negative-phase tank and cuirass-type respirators. 1
2
3
REPORT
6
"From the Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Alberta, Edmonton. * "Resident in Medicine, University of Alberta Hospital, Edmonton. fAssistant Professor of Medicine. {Clinical Head, Department of Medicine, Edmonton General Hospital. §Associate Professor of Medicine and Director, Intensive Care Unit.
2
2
Reprint requests: Dr. King, 6-106 Clinical Sciences Building, University of Alberta, Edmonton
7 8
3 0
% (L/min)
2 5 20 1510-
1
3 0
3 9
4 8
57 •
• CHEST, 76: 2, AUGUST, 1979
(
66
1
75
PA02
(mmHg)
Pa02
(mmHg)
8 4
9 3
102
111
F I G U R E 1. Effects of lowering alveolar oxygen pressure ( P A O ) on ventilation ( V E ) (hypoxic response). Four samples of arterial blood were taken toward end of rebreathing period, and these show hypoxemia. Pa0 , Arterial oxygen pressure. z
—i—
- 1 —
120
2
PRIMARY ALVEOLAR HYPOVENTILATION
219
20
VE
(L/min)
system, such as encephalitis, meningitis, and syringomyelia, was present in some patients. In our patient, extensive neurologic investigation did not reveal any evidence of disease of the central nervous system. Therapy with several drugs, such as dextroamphetamine, aspirin, acetazolamide, progesterone, and theophylline, has been tried in the past in attempts to stimulate chemoreceptor ventilatory drive in such patients, all without significant effect. A thiamine inhibitor was found in two patients with primary alveolar hypoventilation, and administration of thiamine was effective in abolishing the episodes of apnea in one patient. Our patient did not show any response to therapy with progesterone, imipramine, or thiamine. Patients with the syndrome of sleep-related apnea associated with obstruction of the upper airway may have symptoms of snoring, abnormal motor activity during sleep, excessive daytime sleepiness, changes in personality, deteriorating intellectual capacity, hypertension, sexual problems, abnormal outbursts, hypnagogic hallucinations and automatic behavior, and nocturnal enuresis. Various causes for the obstruction have been implicated, ie, a thick short neck, large jowl, hypertrophic palate, and enlarged adenoids or tonsils. Recent evidence suggests that the specific site of obstruction of the airway is in the oral pharynx at the velopharyngeal sphincter. In our patient, clinical examination did not reveal any obvious cause of obstruction; however, after a tonsillectomy and adenoidectomy, the episodes of obstructive apnea could no longer be demonstrated. Hemodynamic studies done in patients with the syndrome of sleep-related apnea have shown cyclic elevations of systemic as well as pulmonary arterial pressures coinciding with the episodes of apnea. Hypoxemia and acidosis were thought to be the important causative factors. These patients may eventually develop sustained pulmonary hypertension and cor pulmonale. Tracheostomy has been found to be effective in abolishing the obstructive type of apnea, although the central apnea may persist. The coexistence of primary alveolar hypoventilation and sleep-associated obstruction of the upper airway was reported recently, and these patients were managed with electrophrenic pacing and tracheostomy. Although experience with electrophrenic pacing is accumulating, success is not universal and depends on careful selection of patients, meticulous technique in implanting the electrodes, and proper use of the apparatus. Tracheostomy 9
10
10
10
11
11
V.
— i —
46
— I —
54
50
58
1—
r—i—
62
66
70
(mmHg) PC0 F I G U R E 2. Effects of increasing carbon dioxide pressure on ventilation ( V E ) (hypercapnic response). Pco , End-tidal carbon dioxide pressure. 2
2
The father, mother, and sister had normal hypoxic and hypercapnic responses. Sleep monitoring was performed, with simultaneous recordings of ear oximetric data, arterial blood gas levels, electroencephalogram, esophageal balloon pressure, and airflowat the nose (monitored by thermistor). These studies documented multiple episodes of apnea throughout the night, some lasting over 90 seconds. Both central and obstructive types of apnea were identified (Fig 3). Recordings of the EEG showed that the patient's pattern of sleep was abnormal, with frequent arousal and predominating light stages of sleep. Rapid-eye-movement sleep was not observed over two nights. Therapy with various medications was tried, including medroxyprogesterone 17-acetate (Provera), imipramine, and thiamine, without any effect Although clinically there was no marked tonsillar enlargement, tonsillectomy and adenoidectomy were performed. On repetition of sleep monitoring, the episodes of obstructive apnea were no longer demonstrable, while episodes of central apnea persisted. At present the patient is managing very well at home, being mechanically ventilated at night with a tank respirator (Emerson). During the day, she functions well without ventilatory assistance, is able to attend school, and has no respiratory difficulties. When travelling, the patient sleeps in a cuirass respirator. DISCUSSION
9
SPONTANEOUS RESPIRATION
11
13
14
15
Severinghaus and Mitchell first used the term, "Ondine's curse," in 1972 in a report describing three patients with loss of automatic control of respiration due to surgical damage to the upper cervical cord; however, the term, "primary alveolar hypoventilation," is now preferred. Mellins et al reviewed 30 reported cases in 1970. The cause of the absent chemical drive was largely obscure, but a history of a disease of the central nervous 1
12
CENTRAL APNOEA
8
16
OBSTRUCTIVE APNOEA
Nasal Thermistor
Esophageal Balloon F I G U R E 3. Representative tracings of nasal temperature (index of air flow) and esophageal pressure obtained during four-hour period of sleep. Tracings show three types of breathing, ie, spontaneous respiration, central apnea, and obstructive apnea.
220
MAN ET AL
CHEST, 7 6 : 2,
AUGUST,
1979
appears to be often necessary, as accentuation of obstruction of the upper airway may occur with pacing. We are not aware of other patients with primary alveolar hypoventilation who are being maintained with the aid of negative-pressure respirators. Although somewhat physically unwieldy, their operation is simple; and intubation, tracheostomy, or other surgical procedures are not required. Moreover, these devices have allowed our patient to have a comfortable sleep at night and complete freedom during the day. Our experience would suggest that negative-pressure ventilators represent a reasonable conservative alternative to electrophrenic pacing for some patients with primary alveolar hypoventilation. ACKNOWLEDGMENT: We wish to thank the respiratory technicians and nursing staff of the Intensive Care Unit, University of Alberta Hospital, Mr. Allan Wells, Ms. Rhoda Cardwell, and Mrs. Margaret Napier for their kind technical assistance and Dr. John Weil for his valuable advice and comments. REFERENCES
1 Severinghaus JW, Mitchell R: Ondine's curse: Failure of respiratory center automaticity while awake. Clin Res 10:122, 1962 2 Guilleminault C, Tilkian A, Dement WC: The sleep apnea syndromes. Ann Rev Med 27:463-484, 1976 3 Whitcomb ME, Altman M, Clark RW, et al: Central and obstructive sleep apnea: Pulmonary disease conference at the Ohio State University, Columbus. Chest 73:857-860, 1978 4 Strohl KP, Saunders NA, Feldman NT, et al: Obstructive sleep apnea in family members. N Engl J Med 299:969973, 1978 5 Phillipson EA: Control of breathing during sleep. Am Rev Respir Dis 118:909-939, 1978 6 Glenn WWL, Gee JBL, Cole DR, et al: Combined central alveolar hypoventilation and upper airway obstruction. Am J Med 64:50-60,1978 7 Rebuck AS, Campbell EJM: A clinical method for assessing the ventilatory response to hypoxia. Am Rev Respir Dis 109:345-350, 1974 8 Read DJC: A clinical method for assessing the ventilatory response to carbon dioxide. Aust Ann Med 16:20-32, 1967 9 Mellins RB, Balfour HH, Turino GM, et al: Failure of automatic control of ventilation (Ondine's curse). Medicine 49:487-504, 1970 10 Deonna T, Arzynska W, Torrado A: Congenital failure of automatic ventilation (Ondine's curse). J Pediatr 84:710714, 1974 11 Guilleminault C, Eldridge FL, Tilkian A: Sleep apnea syndrome due to upper airway obstruction. Arch Intern Med 137:296-300,1977 12 Lonsdale D, Mercer RD: Primary hypoventilation syndrome. Lancet 2:487, 1972 13 Weitzman D, Pollack CP, Borowiecki B: The hypersomnia sleep apnea syndrome: Site and mechanism of upper airways obstruction. In Guilleminault C, Dement WC (eds): Sleep Apnea Syndromes. New York, Alan R Liss, Inc, 1978, pp 235-248 14 Tilkian AG, Guilleminault C, Schroeder JS: Hemodynamics in sleep-induced apnea. Ann Intern Med 85:714719, 1976 15 Sackner MA, Landa J, Forrest T, et al: Periodic sleep CHEST, 7 6 : 2 , AUGUST, 1 9 7 9
apnea: Chronic sleep deprivation related to intermittent upper airway obstruction and central nervous system disturbance. Chest 67:164-171,1975 16 Glenn WWL: Diaphragm pacing: Present status. PACE 1:357-370,1978
Angiosarcoma of the Heart* Diagnosis by Echocardiography Joseph
S. Panella,
Victor,
M.D.;
M.D.;
Ronald
David C. Hueter,
A.
Milton L. Paige, Semerdjian,
M.D.;
M.D.,
Thomas
F.C.C.P.;
A. and
M.D.
Angiosarcoma of the heart is a rare tumor infrequently diagnosed ante mortem. The clinical presentation is usually that of pericarditis or congestive heart failure. This case report illustrates the contribution of echocardiography in suggesting the diagnosis.
A
ngiosarcoma of the heart is a rare disease which is infrequently diagnosed before autopsy. Radiographic examination is occasionally helpful in suggesting the presence of the malignant neoplasm. Angiograms, pneurnopericardiograms, and routine chest x-ray films have all been reported as useful. " The following case report describes a primary cardiac angiosarcoma in which the chest x-ray film and echocardiogram suggested the presence of a cardiac tumor, which was subsequently confirmed by thoracotomy and autopsy. 1
3
CASE REPORT
A 40-year-old white man had been in good health until July 1977, when he developed sharp substernal pain. Both the chest x-ray film, which showed cardiomegaly, and the electrocardiogram were consistent with the clinical impression of pericarditis. An echocardiogram confirmed the presence of a pericardial effusion. The patient's condition initially responded to therapy with aspirin, but symptoms soon recurred. Upon readmission, an echocardiogram (Fig 1A) demonstrated the persistence of a moderate to large pericardial effusion. The patient's condition again responded to conservative management, but complaints of persistent fatigue, abdominal distention, and tightness in the chest resulted in readmission in December 1977. The chest x-rayfilmrevealed persistent cardiomegaly and diffuse nodular infiltrates. An echocardiogram (Fig IB) no longer showed a typical pericardial effusion, but rather a large echo-free space confined anterior to the free right ventricular wall. In addition, there was now marked thickening of the right ventricular epicardial surface suggestive of a clot or tumor. Because of the atypical clinical course and the unusual radiographic and echocardiographic findings, a diagnostic thoracotomy was performed. At surgery a vascular friable tumor filled the pericardial 'From the Departments of Radiology, Pathology, and Internal Medicine, Evanston Hospital and Northwestern University, Evanston, 111.
Reprint requests: Dr. Paige, Department ton Hospital, Evanston, Illinois 60201
of Radiology,
Evans-
ANGIOSARCOMA OF THE HEART 221