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Total anomalous pulmonary venous connection: Surgical correction in a 66-year-old man
Total Anomalous Pulmonary Venous Connection: Surgical Correction in a 66-Year-Old Man Martin H. McMullan, MD, and F. Earl Fyke 111, MD Departments of Cardiovascular Surgery and Adult Cardiology, Mississippi Baptist Medical Center, Jackson, Mississippi
A cyanotic, debilitated 66-year-old man was referred as an emergency because of embolic arterial occlusion of the left lower extremity. Subsequent investigation revealed total anomalous pulmonary venous connection. Complete surgical correction was accomplished. Despite a difficult postoperative hospital course, more than 3
years after operation he continues to experience improved quality of life with better exercise tolerance, freedom from further emboli and symptomatic arrhythmias, and no need for his previously routine phlebotomies. (Ann Thorac Surg 1992;53:520-2)
A
raphy with color flow Doppler examination showed normal left ventricular size and function with paradoxical ventricular septal motion, enlargement of the right atrium and ventricle, a large secundum atrial septal defect with bidirectional shunting, and a dilated coronary sinus that received the pulmonary veins. Arterial oxygen tension on room air was 46 mm Hg. At catheterization, pulmonary artery pressure was 29/18 mm Hg and pulmonary vascular resistance was 160 dynes s * ~ m - ~ Flows . [6] were as follows: pulmonary, 7.0 Wmin; systemic, 4.7 L/min; and effective, 2.4 Llmin. On the eleventh hospital day, repair was performed through a median sternotomy. After institution of cold blood cardioplegia, the right atrium was opened. The common tissue separating the coronary sinus and the left atrium was incised, and the margins were closed with a continuous suture. In the manner described by Reardon and associates [7], a Dacron baffle was inserted so as to close the atrial septal defect and to direct coronary sinus blood flow, and consequently pulmonary venous return, into the left atrium. Initially, mental obtundation complicated postoperative management. Copious pulmonary secretions and associated coughing led to sternal dehiscence necessitating reclosure and to two periods of reintubation. Anticoagulation with warfarin, begun because paroxysms of atrial flutter raised fears of further systemic emboli, was followed by an episode of massive gastrointestinal bleeding. Warfarin administration was discontinued. By the 30th postoperative day, he had recovered adequately to be discharged, his only medications being digoxin (0.125 mg daily) and propranolol (10 mg twice daily) for control of supraventricular arrhythmias and aspirin (80 mg daily). Over follow-up of 38 months, he has resumed his former activities, has experienced no recurrent embolic events, has a normal hematocrit without further phlebotomies, and reports that his exercise tolerance has greatly improved compared with the period preceding surgical repair. He has had no symptomatic arrhythmias and has remained in sinus rhythm on subsequent electrocardiograms.
lthough representing only a small fraction of those patients with total anomalous pulmonary venous connection, rare individuals survive into adulthood without diagnosis [l-31. The oldest survivor heretofore reported, in whom death occurred at age 62 years, was diagnosed at autopsy [4]. The risk of surgical correction is quite low in children and young adults [5]. However, when diagnosis is made in an elderly, debilitated individual, assessment of operative risk becomes difficult, clear precedent for correction is meager, and the decision to proceed may be a difficult one. A 66-year-old man was referred to us in October 1988 after sudden onset of severe pain and pallor in his left leg below the knee. Examination revealed a frail individual with marked cyanosis and clubbing of the digits. The left lower extremity was pale, cool, and without popliteal or pedal pulses. Past history was significant for cyanosis since birth and for frequent respiratory infections and retarded physical development during childhood. In the fifth decade regular phlebotomies for polycythemia began. Anticoagulation with warfarin had been instituted elsewhere for transient cerebral ischemic events but was discontinued because of nosebleeds. Dipyridamole was administered but produced nausea. Nine days before admission a transient ischemic neurologic event accompanied by a grand ma1 seizure occurred. Episodes of supraventricular tachycardia were occurring with increasing frequency. Referral for specific cardiac diagnosis had not been sought because the patient believed that his age and debilitated condition precluded aggressive intervention. The evening of admission, embolectomy with a Fogarty catheter restored circulation to the left lower extremity. Heparin followed by warfarin anticoagulation was begun. Computed tomographic scanning of the head revealed two areas of prior infarction. Transthoracic echocardiogAccepted for publication Aug 16, 1991. Address reprint requests to Dr Fyke, Suite 302, 1600 N State St, Jackson, MS 39202.
0 1992 by
The Society of Thoracic Surgeons
0003-4975/92/$5.00
Ann Thorac Surg 1992:5352&2
Comment Although sometimes accompanied by other malformations, total anomalous pulmonary venous connection usually occurs, as it did in this patient, as an isolated lesion [3]. The majority of patients with total anomalous pulmonary venous connection die in infancy. However, several features seem to enhance the chances for prolonged survival: a short and unobstructed route of anomalous connection, a large interatrial communication, normal pulmonary vascular resistance, and the absence of major systemic oxygen desaturation [2, 3, 51. This patient fared well in these regards except for the severity of hypoxemia and consequent cyanosis. Surgical correction is unquestionably the treatment of choice for the previously undiagnosed child or young adult in otherwise good health. However, in older individuals there is little direct precedence for repair as the oldest such patient heretofore reported was 55 years old [7]; few patients beyond the age of 30 years have been described. Follow-up showing improved survival or quality of life has not been presented. Interestingly, in a review published well into the era of repair with the use of cardiopulmonary bypass, operation ”was not advised” for an active and apparently otherwise healthy 46-yearold patient with total anomalous pulmonary venous connection despite symptoms similar to those of our patient [2]. The closest analogy to which one might appeal is probably correction of atrial septal defect, in which the results of repair in older patients have been better investigated [8, 91. Responding appropriately to this man’s multiple systemic emboli was problematic. Two sources seemed most likely: the left atrium as a result of paroxysmal supraventricular arrhythmias and the systemic veins through paradoxical embolization. Chronic anticoagulation with warfarin was proscribed by his history. Closure of the atrial septal defect, antiplatelet therapy with low-dose aspirin, and amelioration of the atrial arrhythmias have eliminated embolic events thus far. Fortunately, there has been no recurrent gastrointestinal bleeding despite aspirin therapy.
CASE REPORT MCMULLANAND FYKE ANOMALOUS PULMONARY VENOUS CONNECTION
521
In conclusion, undiagnosed cyanotic congenital heart disease in older patients is unusual in this country. Even when surgical correction appears technically feasible, the decision to proceed may be a complex one for the patient, the patient’s family, and the involved physicians. Risk is increased [9] and postoperative care may be difficult. However, if initial obstacles can be surmounted, relief of symptoms and marked and sustained improvement in life quality may be expected.
References 1. Delisle G, Ando M, Calder AL, et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 1976;91:99-122. 2. Jensen JB, Blount SG. Total anomalous pulmonary venous return: a review and report of the oldest surviving patient. Am Heart J 1971;82:387-407. 3. Burroughs JT, Edwards JE. Total anomalous pulmonary venous connection. Am Heart J 1960;59:913-31. 4. McMmus BM, Luetzeler J, Roberts WC. Total anomalous pulmonary venous connection: survival for 62 years without surgical intervention. Am Heart J 1982;103:298-301. 5. Cook!y DA, Hallman GL, Leachman RD. Total anomalous pulmonary venous drainage: correction with the use of cardiopulmonary bypass in 62 cases. J Thorac Cardiovasc Surg 1966;j1:8&102. 6. Schwartz RS, Holmes DR Jr, Fyke FE 111, Mair DD, Smith HC. Cardiac catheterization and angiography: techniques and structures. In: Giuliani ER, Fuster V, Gersh BJ, McGoon MD, McGoon DC, eds. Cardiology: fundamentals and practice. 2nd ed. St. Louis: Mosby-Year Book, 199135269, 7. Reardon MJ, Cooley DA, Kubrusly L, et al. Total anomalous pulmonary venous return: report of 201 patients treated surgically. Tex Heart Inst J 1985;12:13141. 8. St. John Sutton MG, Tajik AJ, McGoon DC. Long-term postoperative follow-up with secundum atrial septal defect (ASD) over 60 years of age [Abstract]. Am J Cardiol 1979;43:363. 9. Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome after surgical repair of isolated atrial septal defect. N Engl J Med 1990:323:1645-50.
INVITED COMMENTARY The report by McMullan and Fyke shows again that congenital cardiac anomalies can be successfully corrected in adult patients. Although some cardiologists contend that the operative risk does not justify the expected benefit, the decision to repair cardiac anomalies should depend on the physiology of the defect and the extent of the disability, rather than the patient’s age. In a &-yearold patient with low pulmonary vascular resistance, the risk should be no greater than that of repairing a large secundum defect. My colleagues and I have successfully corrected total anomalous pulmonary venous return in 4 patients with cyanosis who were more than 30 years of
age (32, 49, 53, and 55 years). In our patients who underwent repair of tetralogy of Fallot, 29 were older than 30 years; the oldest was 73! Survival of patients born with total anomalous pulmonary venous return depends on several factors. In newborns, obstruction of the venous return is life-threatening, with infracardiac and infradiaphragmatic connections producing the highest mortality rates. The length of the venous connection is also important. In the report by McMullan and Fyke, the patient’s defect was of the paracardiac type, which has the shortest possible connection. This short connection probably accounted for the
A cyanotic, debilitated 66-year-old man was referred as an emergency because of embolic arterial occlusion of the left lower extremity. Subsequent investigation revealed total anomalous pulmonary venous connection. Complete surgical correction was accomplished. Despite a difficult postoperative hospital course, more than 3
years after operation he continues to experience improved quality of life with better exercise tolerance, freedom from further emboli and symptomatic arrhythmias, and no need for his previously routine phlebotomies. (Ann Thorac Surg 1992;53:520-2)
A
raphy with color flow Doppler examination showed normal left ventricular size and function with paradoxical ventricular septal motion, enlargement of the right atrium and ventricle, a large secundum atrial septal defect with bidirectional shunting, and a dilated coronary sinus that received the pulmonary veins. Arterial oxygen tension on room air was 46 mm Hg. At catheterization, pulmonary artery pressure was 29/18 mm Hg and pulmonary vascular resistance was 160 dynes s * ~ m - ~ Flows . [6] were as follows: pulmonary, 7.0 Wmin; systemic, 4.7 L/min; and effective, 2.4 Llmin. On the eleventh hospital day, repair was performed through a median sternotomy. After institution of cold blood cardioplegia, the right atrium was opened. The common tissue separating the coronary sinus and the left atrium was incised, and the margins were closed with a continuous suture. In the manner described by Reardon and associates [7], a Dacron baffle was inserted so as to close the atrial septal defect and to direct coronary sinus blood flow, and consequently pulmonary venous return, into the left atrium. Initially, mental obtundation complicated postoperative management. Copious pulmonary secretions and associated coughing led to sternal dehiscence necessitating reclosure and to two periods of reintubation. Anticoagulation with warfarin, begun because paroxysms of atrial flutter raised fears of further systemic emboli, was followed by an episode of massive gastrointestinal bleeding. Warfarin administration was discontinued. By the 30th postoperative day, he had recovered adequately to be discharged, his only medications being digoxin (0.125 mg daily) and propranolol (10 mg twice daily) for control of supraventricular arrhythmias and aspirin (80 mg daily). Over follow-up of 38 months, he has resumed his former activities, has experienced no recurrent embolic events, has a normal hematocrit without further phlebotomies, and reports that his exercise tolerance has greatly improved compared with the period preceding surgical repair. He has had no symptomatic arrhythmias and has remained in sinus rhythm on subsequent electrocardiograms.
lthough representing only a small fraction of those patients with total anomalous pulmonary venous connection, rare individuals survive into adulthood without diagnosis [l-31. The oldest survivor heretofore reported, in whom death occurred at age 62 years, was diagnosed at autopsy [4]. The risk of surgical correction is quite low in children and young adults [5]. However, when diagnosis is made in an elderly, debilitated individual, assessment of operative risk becomes difficult, clear precedent for correction is meager, and the decision to proceed may be a difficult one. A 66-year-old man was referred to us in October 1988 after sudden onset of severe pain and pallor in his left leg below the knee. Examination revealed a frail individual with marked cyanosis and clubbing of the digits. The left lower extremity was pale, cool, and without popliteal or pedal pulses. Past history was significant for cyanosis since birth and for frequent respiratory infections and retarded physical development during childhood. In the fifth decade regular phlebotomies for polycythemia began. Anticoagulation with warfarin had been instituted elsewhere for transient cerebral ischemic events but was discontinued because of nosebleeds. Dipyridamole was administered but produced nausea. Nine days before admission a transient ischemic neurologic event accompanied by a grand ma1 seizure occurred. Episodes of supraventricular tachycardia were occurring with increasing frequency. Referral for specific cardiac diagnosis had not been sought because the patient believed that his age and debilitated condition precluded aggressive intervention. The evening of admission, embolectomy with a Fogarty catheter restored circulation to the left lower extremity. Heparin followed by warfarin anticoagulation was begun. Computed tomographic scanning of the head revealed two areas of prior infarction. Transthoracic echocardiogAccepted for publication Aug 16, 1991. Address reprint requests to Dr Fyke, Suite 302, 1600 N State St, Jackson, MS 39202.
0 1992 by
The Society of Thoracic Surgeons
0003-4975/92/$5.00
Ann Thorac Surg 1992:5352&2
Comment Although sometimes accompanied by other malformations, total anomalous pulmonary venous connection usually occurs, as it did in this patient, as an isolated lesion [3]. The majority of patients with total anomalous pulmonary venous connection die in infancy. However, several features seem to enhance the chances for prolonged survival: a short and unobstructed route of anomalous connection, a large interatrial communication, normal pulmonary vascular resistance, and the absence of major systemic oxygen desaturation [2, 3, 51. This patient fared well in these regards except for the severity of hypoxemia and consequent cyanosis. Surgical correction is unquestionably the treatment of choice for the previously undiagnosed child or young adult in otherwise good health. However, in older individuals there is little direct precedence for repair as the oldest such patient heretofore reported was 55 years old [7]; few patients beyond the age of 30 years have been described. Follow-up showing improved survival or quality of life has not been presented. Interestingly, in a review published well into the era of repair with the use of cardiopulmonary bypass, operation ”was not advised” for an active and apparently otherwise healthy 46-yearold patient with total anomalous pulmonary venous connection despite symptoms similar to those of our patient [2]. The closest analogy to which one might appeal is probably correction of atrial septal defect, in which the results of repair in older patients have been better investigated [8, 91. Responding appropriately to this man’s multiple systemic emboli was problematic. Two sources seemed most likely: the left atrium as a result of paroxysmal supraventricular arrhythmias and the systemic veins through paradoxical embolization. Chronic anticoagulation with warfarin was proscribed by his history. Closure of the atrial septal defect, antiplatelet therapy with low-dose aspirin, and amelioration of the atrial arrhythmias have eliminated embolic events thus far. Fortunately, there has been no recurrent gastrointestinal bleeding despite aspirin therapy.
CASE REPORT MCMULLANAND FYKE ANOMALOUS PULMONARY VENOUS CONNECTION
521
In conclusion, undiagnosed cyanotic congenital heart disease in older patients is unusual in this country. Even when surgical correction appears technically feasible, the decision to proceed may be a complex one for the patient, the patient’s family, and the involved physicians. Risk is increased [9] and postoperative care may be difficult. However, if initial obstacles can be surmounted, relief of symptoms and marked and sustained improvement in life quality may be expected.
References 1. Delisle G, Ando M, Calder AL, et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 1976;91:99-122. 2. Jensen JB, Blount SG. Total anomalous pulmonary venous return: a review and report of the oldest surviving patient. Am Heart J 1971;82:387-407. 3. Burroughs JT, Edwards JE. Total anomalous pulmonary venous connection. Am Heart J 1960;59:913-31. 4. McMmus BM, Luetzeler J, Roberts WC. Total anomalous pulmonary venous connection: survival for 62 years without surgical intervention. Am Heart J 1982;103:298-301. 5. Cook!y DA, Hallman GL, Leachman RD. Total anomalous pulmonary venous drainage: correction with the use of cardiopulmonary bypass in 62 cases. J Thorac Cardiovasc Surg 1966;j1:8&102. 6. Schwartz RS, Holmes DR Jr, Fyke FE 111, Mair DD, Smith HC. Cardiac catheterization and angiography: techniques and structures. In: Giuliani ER, Fuster V, Gersh BJ, McGoon MD, McGoon DC, eds. Cardiology: fundamentals and practice. 2nd ed. St. Louis: Mosby-Year Book, 199135269, 7. Reardon MJ, Cooley DA, Kubrusly L, et al. Total anomalous pulmonary venous return: report of 201 patients treated surgically. Tex Heart Inst J 1985;12:13141. 8. St. John Sutton MG, Tajik AJ, McGoon DC. Long-term postoperative follow-up with secundum atrial septal defect (ASD) over 60 years of age [Abstract]. Am J Cardiol 1979;43:363. 9. Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome after surgical repair of isolated atrial septal defect. N Engl J Med 1990:323:1645-50.
INVITED COMMENTARY The report by McMullan and Fyke shows again that congenital cardiac anomalies can be successfully corrected in adult patients. Although some cardiologists contend that the operative risk does not justify the expected benefit, the decision to repair cardiac anomalies should depend on the physiology of the defect and the extent of the disability, rather than the patient’s age. In a &-yearold patient with low pulmonary vascular resistance, the risk should be no greater than that of repairing a large secundum defect. My colleagues and I have successfully corrected total anomalous pulmonary venous return in 4 patients with cyanosis who were more than 30 years of
age (32, 49, 53, and 55 years). In our patients who underwent repair of tetralogy of Fallot, 29 were older than 30 years; the oldest was 73! Survival of patients born with total anomalous pulmonary venous return depends on several factors. In newborns, obstruction of the venous return is life-threatening, with infracardiac and infradiaphragmatic connections producing the highest mortality rates. The length of the venous connection is also important. In the report by McMullan and Fyke, the patient’s defect was of the paracardiac type, which has the shortest possible connection. This short connection probably accounted for the