- Email: [email protected]
Carcinoid crisis after biopsy of a bronchial carcinoid
CASE REPORT KARMY-JONES AND V A L L I ~ R E S CARCINOID CRISIS WITH BRONCHIAL CARCINOID
Ann Thorac Surg 1993;56:1403-5
1403
References 1. Wong J, Loyd JE, Merrill WH, Frist WH. Single lung trans-
Fig 2 . Aortobronchial fistula seen from aortic end of fistula. Probe lies in fistulous tract; intercostal arteries are seen distally.
The fistula in our patient may have been secondary to migration of the wire stent with erosion into the aorta. The relative rigidity of wire stents in an environment of constant respiratory and vascular motion would explain this mechanism. The preexisting fungal infection and the absence of cartilaginous support also likely played contributing roles. Based on this hypothesis, we do not recommend the long-term use of expanding or rigid wire stents in cases of major bronchial wall dehiscence. The incidence of airway complications in this population has been reported to be 14% to 30%, with dehiscence occurring in 3% to 15% of patients and stenosis in 6% to 25% [ 2 4 ] . De Hoyos and colleagues reported improved bronchial anastomotic healing using the telescoping technique [3]. Improvements in bronchial microcirculation with the use of intravenous heparin and prostacyclin may also lead to a reduction in anastomotic complications [5]. Dehiscence must be detected early if treatment is to prevent serious complication. Omentopexy has been shown to improve bronchial healing and can often contain a small, noncircumferential dehiscence, which can then be managed nonoperatively [4]. Less commonly, reoperation will be required. Stenosis of the bronchial anastomosis is a more common problem, believed to be most often related to ischemia at the anastomotic site, but it may occur with noncircumferential leaks that heal with exuberant granulation tissue formation. Available endoscopic therapies include flexible bronchoscopy with simple tissue excision, rigid bronchoscopy with mechanical dilation, neodymium:yttrium-aluminum garnet laser ablation and photocoagulation, and placement of endobronchial stents [6]. Stents are commercially available, and considerable experience has developed with their use [7, 81. There are reports of stents left in place for as long as 4.5 years [8]. Transplant surgeons should have at their disposal all of the discussed techniques to manage the challenging airway complications that commonly occur after lung transplantation. Only aggressive surveillance and treatment will ensure continued success with the pulmonary transplantation techniques and technology that currently exist.
plantation: replacement therapy for end-stage lung disease. South Med J 1992;85:887-93. 2. Haydock DA, Trulock EP, Kaiser LR, et al. Lung transplantation. Analysis of thirty-six consecutive procedures performed over a twelve-month period. The Washington University Lung Transplant Group. J Thorac Cardiovasc Surg 1992;103: 32940. 3. De Hoyos AL, Patterson GA, Maurere JR, Ramirez JC, Miller JD, Winton TL. Pulmonary transplantation. Early and late results. The Toronto Lung Transplant Group. J Thorac Cardiovasc Surg 1992;103:295-306. 4. Schafers HJ, Haydock DA, Cooper JD. The prevalence and management of bronchial anastomotic complications in lung transplantation.J Thorac Cardiovasc Surg 1991;101:104452. 5 . Schafers HJ, Haverich A, Wagner TOF, et al. Decreased incidence of bronchial complications following lung transplantation. Eur J Cardiothorac Surg 1992;6:1749. 6. Colt HB, Janssen JP, Dumon JF, Noirclerc MJ. Endoscopic management of bronchial stenosis after double lung transplantation. Chest 1992;102:10-6. 7. Nashef SAM, Dromer C, Velly JF, Labrousse J, Couraud L. Expanding wire stents in benign tracheobronchial disease: indications and complications. Ann Thorac Surg 1992;54: 93740. 8. Cooper JD, Pearson FG, Patterson GA, et al. Use of silicone stents in the management of airway problems. Ann Thorac Surg 1989;47:371-8.
Carcinoid Crisis After Biopsy of a Bronchial Carcinoid Riyad Karmy-Jones,
MD, and Eric Vallieres, MD
Division of Cardiothoracic Surgery, University of Alberta Hospitals, Edmonton, Alberta
Pulmonary carcinoids are rarely associated with carcinoid syndromes and even less commonly with carcinoid crisis. Somatostatin analogues can control carcinoid syndrome or crisis with tumors of gastrointestinal origin. We report the successful use of a somatostatin analogue in preventing carcinoid crisis at the time of resection of an "active" bronchial carcinoid tumor. (Ann Thorac Surg 1993;56:1403-5)
A
46-year-old woman presented with an "asymptomatic" 2-cm nodule in the right lung (Fig 1).Bronchoscopy revealed a typical bronchial adenoma in the superior segmental orifice, right lower lobe. Soon after biopsy, pulmonary edema and vasomotor collapse rapidly developed, requiring intubation, ventilatory and inotropic support for 48 hours. She recovered without sequelae. Pathologic examination revealed a carcinoid tumor. Serum 5-hydroxyindoleacetic acid level measured at the time of crisis was 81 IU/dL (normal, 20 to 50 IU/dL). The patient
Accepted for publication March 24, 1993 Address reprint requests to Dr Vallihres, Thoracic Surgery, University of Alberta Hospitals, 2D4.32 Walter Mackenzie Health Sciences Centre, 8440 112 St, Edmonton, Alberta T6G 287, Canada.
1404
CASE REPORT KARMY-JONESAND VALLIBRES CARCINOID CRISIS WITH BRONCHIAL CARCINOID
Fig I. Plain chest radiograph showing a 2-cm central right lower lobe nodule (arrows).
was referred for resection. On further questioning, she described long-term flushing with red wine intake and exercise, as well as palpitations on lying down. Physical examination and complete metabolic work-up, including serum and urine 5-hydroxyindoleacetic acid levels, were normal. Full metastatic work-up (including meta iodo benzyl guanidine scan and computed tomography) was negative. The patient received 10 days of preoperative therapy at home consisting of three daily subcutaneous 50-pg doses of octreotide, a somatostatin analogue (Sandostatin; Sandoz, Dorval, Que, Canada), and cimetidine, 400 mg orally twice daily, then underwent an uneventful right lower lobectomy. Attention was made to ligating the inferior pulmonary vein before manipulating the tumor. Pathologic examination revealed a 1.7-cm typical T1 NO argyrophilic carcinoid tumor. Electron microscopy showed an abundance of secretory granules within tumoral tissue. Tissue serotonin levels (Serotonin Assay; Bio/Can Scientific, Mississauga, Ont, Canada) were measured at 283 nmol/g of tumor (4.2 nmol/g in normal lung tissue). (Levels on two control nonfunctioning carcinoid tumors resected since were measured at 11.77 and 8.74 nmoYg of tumor, respectively.) The patient was discharged 5 days postoperatively and remains well at 2 years follow-up. Preoperative flushing and palpitations have not recurred.
Comment Carcinoid tumors may secrete a variety of vasopeptides, of which histamine and serotonin are believed to cause the carcinoid syndrome [ 1-81. The syndrome typically occurs when degrading enzymatic systems in liver or lung
Ann Thorac Surg 1993;56140>5
are bypassed or overwhelmed [l-61. Carcinoid tumors may give rise to a carcinoid crisis, which can take the form of severe hypertension, flushing, and acidosis due to serotonin release or hypotension, diarrhea, and bronchoconstriction attributable to kallikrein or histamine release (1, 4, 51. Crisis may be precipitated by several factors including manipulation of the tumor [4-61. Crisis are rare, with one series noting only six episodes in 410 patients undergoing abdominal exploration for carcinoid tumors 141. Bronchopulmonary carcinoid tumors may present with hemoptysis, postobstructive pneumonitis, or unilateral wheeze [3], or may be found on a routine chest roentgenograms in asymptomatic patients [7]. Prognosis is affected by an asymptomatic presentation, the histology of the tumor, and the lymph node status [2, 71. The incidence of carcinoid syndrome is 2% to 12%[2, 71, and the syndrome as well as the crisis are rare in the absence of spread [2-6]. Review of recent series revealed that only 2 of the 74 patients with bronchial carcinoid syndrome were free of metastases [2, 3, 61. Carcinoid crisis has never been reported with primary lung tumors in the absence of either locally invasive large tumors or hepatic metastases, which may occur years after initial resection of the primary lung tumor [2, 61. Perioperative management of patients with carcinoid tumors includes the avoidance of known precipitating agents, proper hydration, and the use of cimetidine to inhibit histamine release [l,4, 51. Somatostatin naturally inhibits the release of a variety of peptides, including serotonin, by blocking calcium channels [4]. In carcinoids, it diminishes the synthesis and secretion of serotonin via a second messenger system [8]. Long-acting somatostatin analogues have been used as prophylaxis for and treatment of both carcinoid syndromes [ 4 7 ] and crises of gastrointestinal origin. The association of bronchopulmonary carcinoid with carcinoid syndrome is uncommon and should prompt a work-up to rule out systemic metastases or local spread. Patients who present for resection and who have manifestations of carcinoid syndrome should be considered for preoperative prophylaxis with a somatostatin analogue and cimetidine. Somatostatin analogue may be life-saving in the rare event of carcinoid crisis associated with bronchopulmonary carcinoids.
References 1. Tilson MD. Carcinoid syndrome. Surg Clin N Am 1974;54 409-23. 2. Ricci C, Patrassi N, Massa R, Mineo C, Benedetti-Valentini F. Carcinoid syndrome in bronchial adenoma. Am J Surg 1973; 126~671-7. 3. Todd TR, Cooper JD, Weissberg D, Delarue ND, Pearson FG. Bronchial carcinoid tumors. J Thorac Cardiovasc Surg 1980;79: 532-6. 4. Marsh HM, Martin JK Jr, Kvols LK, et al. Carcinoid crisis during anesthesia: successful treatment with a somatostatin analogue. Anesthesiology 1987;66:89-91. 5. Oates JA. The carcinoid syndrome. N Engl J Med 1986;ll: 702-4. 6. Hughes EW, Hodkinson BP. Carcinoid syndrome: the combined use of ketanserin and octreotide in the management of
Ann Thorac Surg 1993;5614056
an acute crisis during anesthesia. Anaesth Intensive Care 1989;17367-9. 7. Harpole DH, Feldman JM, Buchanan S, et al. Bronchial carcinoid tumors: a retrospective analysis of 126 patients. Ann Thorac Surg 1992;54:50-5. 8. Wanabert B, Nilsson 0, Theodorsson E, Dahlstrom A, Ahlman H. The effect of a somatostatin analogue on the release of hormones from human midgut carcinoid tumor cells. Br J Cancer 1991;64:234.
Congenital Absence of the Pericardium: Displacement of the Heart Associated With Tricuspid Insufficiency Jacques A. M. van Son, MD, PhD, Gordon K. Danielson, MD, and John A. Callahan, MD Divisions of Thoracic and Cardiovascular Surgery and Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota
This report describes a patient with congenital absence of the left pericardium with displacement of the heart into the left pleural space and associated tricuspid insufficiency. Excision of the ruptured chordae, plication of the flail anterior leaflet, and tricuspid annuloplasty rendered a competent tricuspid valve. (Ann Tkorac Surg 1993;56:1405-6)
T
he vast majority of pericardial defects occur on the left side and are thought to be due to premature atrophy of the left common cardinal vein or duct of Cuvier [l]. Moderate-sized defects are most likely to produce symptoms because of their potential for cardiac herniation and strangulation [2-61. Although unilateral complete absence of the pericardium is rarely of clinical importance [7, 81, we report the clinical course and surgical treatment of a severely symptomatic patient who had complete absence of the left pericardium. A 42-year-old man was admitted because of severe dyspnea and peripheral edema. The chest radiogram showed marked cardiomegaly and leftward displacement of the heart. The electrocardiogram demonstrated normal sinus rhythm with marked left axis deviation. A flail anterior leaflet of the tricuspid valve with systolic prolapse into the right atrium was seen by two-dimensional echocardiography; the mitral and aortic valves were normal. Angiography showed severe tricuspid valve regurgitation and marked dilation of the right atrium and right ventricle. The coronary arteries were normal. The patient had a history of rheumatic fever and motor vehicle accident 32 and 20 years before the operation, respectively. At operation, the left pericardium was completely abAccepted for publication March 25, 1993 Address reprint requests to Dr Danielson, Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905.
CASE REPORT VAN SON ET AL CONGENITAL ABSENCE OF PERICARDIUM
1405
sent. The left phrenic neurovascular bundle coursed in the anterior edge of the defect. The heart was lying entirely in the left chest with the apex touching the posterior portion of the left pleural space. The only portion of the heart visible from the median sternotomy was the right atrium and aorta (Fig 1).The right ventricle was pulling on the right atrium, which was markedly attenuated. After cardiopulmonary bypass had been instituted, the right atrium was opened and the tricuspid valve was inspected. A flail anterior leaflet with rupture of several chordae was found (Fig 2 ) . There was leaflet retraction where the chordae were ruptured and the annulus was markedly dilated. The ruptured chordae were excised and the anterior leaflet was plicated so as to obliterate the tissue defect (Fig 3). Chordae were then present along the entire free edge of the leaflet. In addition, a de Vega annuloplasty was performed. The tricuspid valve was tested with saline solution injected forcibly into the right ventricle and was found to be competent. To minimize the possibility of recurrence of tricuspid insufficiency, we made an attempt to repair the pericardial defect by closing it with two large dural patches (glutaraldehyde-preserved bovine pericardial patches were not available at the time of this operation). When the sternum was approximated, however, it was seen that there was significant compromise of cardiac function because of the small anterior mediastinum. Consequently, it was necessary to remove the dural patches and replace the heart in the left pleural space. The heart remained in normal sinus rhythm after the procedure. The patient had an uneventful postoperative recovery,
Fig 1 . Intraoperative surgeon's view of absent left pericardium. The only structures of the heart visible from the median sternotomy are the bulging right atrium (RA), the ascending aorta (white arrowhead), and a small part of the right ventricle (black arrowhead). Note the communication with the left pleural space. (L = left lung.)
Ann Thorac Surg 1993;56:1403-5
1403
References 1. Wong J, Loyd JE, Merrill WH, Frist WH. Single lung trans-
Fig 2 . Aortobronchial fistula seen from aortic end of fistula. Probe lies in fistulous tract; intercostal arteries are seen distally.
The fistula in our patient may have been secondary to migration of the wire stent with erosion into the aorta. The relative rigidity of wire stents in an environment of constant respiratory and vascular motion would explain this mechanism. The preexisting fungal infection and the absence of cartilaginous support also likely played contributing roles. Based on this hypothesis, we do not recommend the long-term use of expanding or rigid wire stents in cases of major bronchial wall dehiscence. The incidence of airway complications in this population has been reported to be 14% to 30%, with dehiscence occurring in 3% to 15% of patients and stenosis in 6% to 25% [ 2 4 ] . De Hoyos and colleagues reported improved bronchial anastomotic healing using the telescoping technique [3]. Improvements in bronchial microcirculation with the use of intravenous heparin and prostacyclin may also lead to a reduction in anastomotic complications [5]. Dehiscence must be detected early if treatment is to prevent serious complication. Omentopexy has been shown to improve bronchial healing and can often contain a small, noncircumferential dehiscence, which can then be managed nonoperatively [4]. Less commonly, reoperation will be required. Stenosis of the bronchial anastomosis is a more common problem, believed to be most often related to ischemia at the anastomotic site, but it may occur with noncircumferential leaks that heal with exuberant granulation tissue formation. Available endoscopic therapies include flexible bronchoscopy with simple tissue excision, rigid bronchoscopy with mechanical dilation, neodymium:yttrium-aluminum garnet laser ablation and photocoagulation, and placement of endobronchial stents [6]. Stents are commercially available, and considerable experience has developed with their use [7, 81. There are reports of stents left in place for as long as 4.5 years [8]. Transplant surgeons should have at their disposal all of the discussed techniques to manage the challenging airway complications that commonly occur after lung transplantation. Only aggressive surveillance and treatment will ensure continued success with the pulmonary transplantation techniques and technology that currently exist.
plantation: replacement therapy for end-stage lung disease. South Med J 1992;85:887-93. 2. Haydock DA, Trulock EP, Kaiser LR, et al. Lung transplantation. Analysis of thirty-six consecutive procedures performed over a twelve-month period. The Washington University Lung Transplant Group. J Thorac Cardiovasc Surg 1992;103: 32940. 3. De Hoyos AL, Patterson GA, Maurere JR, Ramirez JC, Miller JD, Winton TL. Pulmonary transplantation. Early and late results. The Toronto Lung Transplant Group. J Thorac Cardiovasc Surg 1992;103:295-306. 4. Schafers HJ, Haydock DA, Cooper JD. The prevalence and management of bronchial anastomotic complications in lung transplantation.J Thorac Cardiovasc Surg 1991;101:104452. 5 . Schafers HJ, Haverich A, Wagner TOF, et al. Decreased incidence of bronchial complications following lung transplantation. Eur J Cardiothorac Surg 1992;6:1749. 6. Colt HB, Janssen JP, Dumon JF, Noirclerc MJ. Endoscopic management of bronchial stenosis after double lung transplantation. Chest 1992;102:10-6. 7. Nashef SAM, Dromer C, Velly JF, Labrousse J, Couraud L. Expanding wire stents in benign tracheobronchial disease: indications and complications. Ann Thorac Surg 1992;54: 93740. 8. Cooper JD, Pearson FG, Patterson GA, et al. Use of silicone stents in the management of airway problems. Ann Thorac Surg 1989;47:371-8.
Carcinoid Crisis After Biopsy of a Bronchial Carcinoid Riyad Karmy-Jones,
MD, and Eric Vallieres, MD
Division of Cardiothoracic Surgery, University of Alberta Hospitals, Edmonton, Alberta
Pulmonary carcinoids are rarely associated with carcinoid syndromes and even less commonly with carcinoid crisis. Somatostatin analogues can control carcinoid syndrome or crisis with tumors of gastrointestinal origin. We report the successful use of a somatostatin analogue in preventing carcinoid crisis at the time of resection of an "active" bronchial carcinoid tumor. (Ann Thorac Surg 1993;56:1403-5)
A
46-year-old woman presented with an "asymptomatic" 2-cm nodule in the right lung (Fig 1).Bronchoscopy revealed a typical bronchial adenoma in the superior segmental orifice, right lower lobe. Soon after biopsy, pulmonary edema and vasomotor collapse rapidly developed, requiring intubation, ventilatory and inotropic support for 48 hours. She recovered without sequelae. Pathologic examination revealed a carcinoid tumor. Serum 5-hydroxyindoleacetic acid level measured at the time of crisis was 81 IU/dL (normal, 20 to 50 IU/dL). The patient
Accepted for publication March 24, 1993 Address reprint requests to Dr Vallihres, Thoracic Surgery, University of Alberta Hospitals, 2D4.32 Walter Mackenzie Health Sciences Centre, 8440 112 St, Edmonton, Alberta T6G 287, Canada.
1404
CASE REPORT KARMY-JONESAND VALLIBRES CARCINOID CRISIS WITH BRONCHIAL CARCINOID
Fig I. Plain chest radiograph showing a 2-cm central right lower lobe nodule (arrows).
was referred for resection. On further questioning, she described long-term flushing with red wine intake and exercise, as well as palpitations on lying down. Physical examination and complete metabolic work-up, including serum and urine 5-hydroxyindoleacetic acid levels, were normal. Full metastatic work-up (including meta iodo benzyl guanidine scan and computed tomography) was negative. The patient received 10 days of preoperative therapy at home consisting of three daily subcutaneous 50-pg doses of octreotide, a somatostatin analogue (Sandostatin; Sandoz, Dorval, Que, Canada), and cimetidine, 400 mg orally twice daily, then underwent an uneventful right lower lobectomy. Attention was made to ligating the inferior pulmonary vein before manipulating the tumor. Pathologic examination revealed a 1.7-cm typical T1 NO argyrophilic carcinoid tumor. Electron microscopy showed an abundance of secretory granules within tumoral tissue. Tissue serotonin levels (Serotonin Assay; Bio/Can Scientific, Mississauga, Ont, Canada) were measured at 283 nmol/g of tumor (4.2 nmol/g in normal lung tissue). (Levels on two control nonfunctioning carcinoid tumors resected since were measured at 11.77 and 8.74 nmoYg of tumor, respectively.) The patient was discharged 5 days postoperatively and remains well at 2 years follow-up. Preoperative flushing and palpitations have not recurred.
Comment Carcinoid tumors may secrete a variety of vasopeptides, of which histamine and serotonin are believed to cause the carcinoid syndrome [ 1-81. The syndrome typically occurs when degrading enzymatic systems in liver or lung
Ann Thorac Surg 1993;56140>5
are bypassed or overwhelmed [l-61. Carcinoid tumors may give rise to a carcinoid crisis, which can take the form of severe hypertension, flushing, and acidosis due to serotonin release or hypotension, diarrhea, and bronchoconstriction attributable to kallikrein or histamine release (1, 4, 51. Crisis may be precipitated by several factors including manipulation of the tumor [4-61. Crisis are rare, with one series noting only six episodes in 410 patients undergoing abdominal exploration for carcinoid tumors 141. Bronchopulmonary carcinoid tumors may present with hemoptysis, postobstructive pneumonitis, or unilateral wheeze [3], or may be found on a routine chest roentgenograms in asymptomatic patients [7]. Prognosis is affected by an asymptomatic presentation, the histology of the tumor, and the lymph node status [2, 71. The incidence of carcinoid syndrome is 2% to 12%[2, 71, and the syndrome as well as the crisis are rare in the absence of spread [2-6]. Review of recent series revealed that only 2 of the 74 patients with bronchial carcinoid syndrome were free of metastases [2, 3, 61. Carcinoid crisis has never been reported with primary lung tumors in the absence of either locally invasive large tumors or hepatic metastases, which may occur years after initial resection of the primary lung tumor [2, 61. Perioperative management of patients with carcinoid tumors includes the avoidance of known precipitating agents, proper hydration, and the use of cimetidine to inhibit histamine release [l,4, 51. Somatostatin naturally inhibits the release of a variety of peptides, including serotonin, by blocking calcium channels [4]. In carcinoids, it diminishes the synthesis and secretion of serotonin via a second messenger system [8]. Long-acting somatostatin analogues have been used as prophylaxis for and treatment of both carcinoid syndromes [ 4 7 ] and crises of gastrointestinal origin. The association of bronchopulmonary carcinoid with carcinoid syndrome is uncommon and should prompt a work-up to rule out systemic metastases or local spread. Patients who present for resection and who have manifestations of carcinoid syndrome should be considered for preoperative prophylaxis with a somatostatin analogue and cimetidine. Somatostatin analogue may be life-saving in the rare event of carcinoid crisis associated with bronchopulmonary carcinoids.
References 1. Tilson MD. Carcinoid syndrome. Surg Clin N Am 1974;54 409-23. 2. Ricci C, Patrassi N, Massa R, Mineo C, Benedetti-Valentini F. Carcinoid syndrome in bronchial adenoma. Am J Surg 1973; 126~671-7. 3. Todd TR, Cooper JD, Weissberg D, Delarue ND, Pearson FG. Bronchial carcinoid tumors. J Thorac Cardiovasc Surg 1980;79: 532-6. 4. Marsh HM, Martin JK Jr, Kvols LK, et al. Carcinoid crisis during anesthesia: successful treatment with a somatostatin analogue. Anesthesiology 1987;66:89-91. 5. Oates JA. The carcinoid syndrome. N Engl J Med 1986;ll: 702-4. 6. Hughes EW, Hodkinson BP. Carcinoid syndrome: the combined use of ketanserin and octreotide in the management of
Ann Thorac Surg 1993;5614056
an acute crisis during anesthesia. Anaesth Intensive Care 1989;17367-9. 7. Harpole DH, Feldman JM, Buchanan S, et al. Bronchial carcinoid tumors: a retrospective analysis of 126 patients. Ann Thorac Surg 1992;54:50-5. 8. Wanabert B, Nilsson 0, Theodorsson E, Dahlstrom A, Ahlman H. The effect of a somatostatin analogue on the release of hormones from human midgut carcinoid tumor cells. Br J Cancer 1991;64:234.
Congenital Absence of the Pericardium: Displacement of the Heart Associated With Tricuspid Insufficiency Jacques A. M. van Son, MD, PhD, Gordon K. Danielson, MD, and John A. Callahan, MD Divisions of Thoracic and Cardiovascular Surgery and Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota
This report describes a patient with congenital absence of the left pericardium with displacement of the heart into the left pleural space and associated tricuspid insufficiency. Excision of the ruptured chordae, plication of the flail anterior leaflet, and tricuspid annuloplasty rendered a competent tricuspid valve. (Ann Tkorac Surg 1993;56:1405-6)
T
he vast majority of pericardial defects occur on the left side and are thought to be due to premature atrophy of the left common cardinal vein or duct of Cuvier [l]. Moderate-sized defects are most likely to produce symptoms because of their potential for cardiac herniation and strangulation [2-61. Although unilateral complete absence of the pericardium is rarely of clinical importance [7, 81, we report the clinical course and surgical treatment of a severely symptomatic patient who had complete absence of the left pericardium. A 42-year-old man was admitted because of severe dyspnea and peripheral edema. The chest radiogram showed marked cardiomegaly and leftward displacement of the heart. The electrocardiogram demonstrated normal sinus rhythm with marked left axis deviation. A flail anterior leaflet of the tricuspid valve with systolic prolapse into the right atrium was seen by two-dimensional echocardiography; the mitral and aortic valves were normal. Angiography showed severe tricuspid valve regurgitation and marked dilation of the right atrium and right ventricle. The coronary arteries were normal. The patient had a history of rheumatic fever and motor vehicle accident 32 and 20 years before the operation, respectively. At operation, the left pericardium was completely abAccepted for publication March 25, 1993 Address reprint requests to Dr Danielson, Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905.
CASE REPORT VAN SON ET AL CONGENITAL ABSENCE OF PERICARDIUM
1405
sent. The left phrenic neurovascular bundle coursed in the anterior edge of the defect. The heart was lying entirely in the left chest with the apex touching the posterior portion of the left pleural space. The only portion of the heart visible from the median sternotomy was the right atrium and aorta (Fig 1).The right ventricle was pulling on the right atrium, which was markedly attenuated. After cardiopulmonary bypass had been instituted, the right atrium was opened and the tricuspid valve was inspected. A flail anterior leaflet with rupture of several chordae was found (Fig 2 ) . There was leaflet retraction where the chordae were ruptured and the annulus was markedly dilated. The ruptured chordae were excised and the anterior leaflet was plicated so as to obliterate the tissue defect (Fig 3). Chordae were then present along the entire free edge of the leaflet. In addition, a de Vega annuloplasty was performed. The tricuspid valve was tested with saline solution injected forcibly into the right ventricle and was found to be competent. To minimize the possibility of recurrence of tricuspid insufficiency, we made an attempt to repair the pericardial defect by closing it with two large dural patches (glutaraldehyde-preserved bovine pericardial patches were not available at the time of this operation). When the sternum was approximated, however, it was seen that there was significant compromise of cardiac function because of the small anterior mediastinum. Consequently, it was necessary to remove the dural patches and replace the heart in the left pleural space. The heart remained in normal sinus rhythm after the procedure. The patient had an uneventful postoperative recovery,
Fig 1 . Intraoperative surgeon's view of absent left pericardium. The only structures of the heart visible from the median sternotomy are the bulging right atrium (RA), the ascending aorta (white arrowhead), and a small part of the right ventricle (black arrowhead). Note the communication with the left pleural space. (L = left lung.)