Postpericardiotomy syndrome following surgery for congenital disease

The postpericardiotomy syndrome (PPS) may occur after intrapericardial surgery and is usually characterized by and pericardial and pleural Derek Fyfe, M.D., Ph.D. effusions as major clinical manifestations.1-3 A sim- ilar syndrome has also been observed after myocar- Department of Pediatrics and Adolescent 4 Medicine dial infarction (Dressler's syndrome), and after penetrating or blunt trauma to the .5 Douglas S. Moodie, M.D. As yet no specific treatment for this syndrome is available and anti-inflammatory agents with sup- Department of portive medical or surgical measures are generally Department of Pediatrics and Adolescent Medicine used when necessary. Although PPS is believed to be a self-limited illness, in some cases it has led to Carl C. Gill, M.D. and death. In the largest re- ported series of 400 patients who had undergone Department of Thoracic and Cardiovascular intracardiac surgery, PPS occurred in about 27%; Surgery it rarely occurs before age 2. The recent surgical experience with congenital heart disease at the Cleveland Clinic was reviewed with regard to the frequency and manifestations of the clinically significant PPS.

Results PPS occurred in 13 of 217 patients after correc- tion of various congenital cardiac lesions (Table). Six of 13 patients had simple secundum atrial septal defects. The other congenital lesions are listed in the Table. The age range was 11 to 47 years (mean,

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21.6 years). Nine of the 13 patients were diograms demonstrated large pericar- between 11 and 20 years of age. The dial effusions in all 13 patients. Electro- most frequent presenting symptoms cardiographic findings of ST elevation were fever, pleuritic , and were present in only two patients. Chest shoulder pain (eight patients). Frequent roentgenograms demonstrated in- findings on physical examination were creased cardiac size in all patients and decreased breath sounds (eight pa- pleural effusions in six patients. Labo- tients), and the presence of a pleural or ratory studies including white blood cell pericardial rub (six patients). Symptoms counts (WBC) revealed only moderate began between the third and 55th days elevations of leukocytes usually accom- postoperatively; the mean onset was 16 panied by a predominance of neutro- days, and the mean duration of the phils. Several blood cultures done on syndrome was 29 days. Initial echocar- each patient were negative.

Fig. 1. Preoperative chest roentgenogram before of an anomalous right ventricular outflow tract muscle bundle.

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Four of the 13 patients had moderate Medications prescribed were aspirin, to severe disease. Two patients required indomethacin, and prednisone Five pa- thoracentesis, and one patient required tients were receiving warfarin sodium emergency pericardiocentesis for tam- (Coumadin) at onset of PPS. Five of the ponade and resection of a pericardial 13 patients were rehospitalized with window. In this patient, 1000 cc of ser- PPS. There were no deaths in this series. ous pericardial fluid was aspirated and the patient subsequently had a cardiac Discussion arrest 12 hours postoperatively. After 2 In our experience with clinically sig- days of continuous inotropic support, nificant PPS after surgery for congenital the patient completely recovered. The heart disease, onset of symptoms tended progression of roentgenographic to be within the first 2 weeks postoper- changes is shown in Figures l-4\ a large atively, often heralded by fever, pleuri- posterior was shown tic chest pain, and cough. Patients usu- by echocardiography (Fig. 5). ally had decreased breath sounds and a

Fig. 2. Postoperative day 15 showing severe and large bilateral pleural effusions.

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Fig. 3. Postoperative day 23 showing prominent globular heart and resolving pleural effusions following treatment with anti-inflammatory agents. pleural or pericardial rub by examina- was significantly less in patients older tion. All patients had large pericardial than 40 years of age and rare in patients and often pleural effusions on initial younger than age 2. In the present re- chest roentgenogram and echocardio- port, the 6% incidence may reflect the gram. Neither electrocardiograms nor fact that most of our patients with PPS WBCs were helpful in diagnosis or in had clinically significant illness, causing following the course of the disease. The prolonged hospitalization or rehospital- severity of clinical symptoms ranged ization in some cases. The symptoms of from mild in most cases, to severe in fever, pain, and polyserositis used as three patients, one of whom developed diagnostic criteria by most observers tamponade and suffered . were not uniformly present in our pa- All patients survived. tients, although all had moderate to A 6%7 to 53% incidence of PPS has large pericardial effusions. In a study by 8 been reported.8 Recently, in a series of Clapp et ai, diagnostic criteria were 400 pediatric patients, Engle et ale re- liberalized, i.e., patients were selected ported an overall incidence of 27%. This by the presence of three of five clinical

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Fig. 4. Postoperative follow-up chest roentgenogram at 6 months. features: fever, chest pain, pericardial cardiograms are not routinely obtained friction rub, fluid retention, and irrita- following . bility or anorexia. Sixteen of 18 patients It is important to study the relation- with PPS as defined above had echocar- ship between early pericardial effusions diography findings of pericardial effu- and immunologic factors to determine sion, and in 15 patients, pericardial ef- those features associated with different fusion was present by the second post- clinical outcomes. It is possible that the operative day. Only one patient with mild early disease may be different from pericardial effusion had no clinical evi- the lingering, life-threatening disease dence of PPS. This specific finding may, seen in some of our patients. therefore, indicate that PPS or "benign It has been demonstrated that pa- postoperative " may be un- tients with PPS may have increased ti- derdiagnosed when postoperative echo- ters of antibodies to one or even several

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Fig. 5. Echocardiogram showing large posterior pericardial effusion from the same patient.

viruses that are epidemic in the com- ular septal defect repair when infun- munity at or after the time of surgery, dibulectomy was also performed, i.e., Coxsackie, adenovirus, and cyto- whereas when simple ventricular septal megaloviruses. Also, a putative "anti- defect was corrected, the incidence was heart antibody" may also be present in only 9%. However, atrial septal defect high titer in the sera of patients with repair, in which no muscle resection is PPS. Engle et al3 postulated that PPS required, accounted for 24% of patients. may represent an autoimmune disease Also, in the series reported here, atrial triggered by a viral infection after peri- septal defect was often associated with cardial or myocardial injury. This at- PPS, again casting doubt on this hy- tractive hypothesis fails, however, to en- pothesis. compass findings of elevated antiheart As a clinical entity, PPS is not uncom- antibodies in patients postoperatively or mon and may be mild or life threaten- in those who have ing. It is apparent that identification of when no PPS is present.4,9, 10 Antiheart more specific biochemical or immuno- antibodies may represent an epipheno- logic markers is needed before the full menon, being formed in response to spectrum of PPS is comprehensible and myocardial damage due to various optimal therapy may be provided. mechanisms but not etiologic to PPS itself. References It has also been suggested that devel- 1. Ito T, Engle MA, Goldberg HP. Postpericar- opment of PPS may relate to the extent diotomy syndrome following surgery for non- rheumatic heart disease. Circulation 1958; 17: of surgical manipulation of the myocar- 549-56. 11 3 dium. In the series of Engle et al, 40% 2. Engle MA, Ito T. The postpericardiotomy of patients had PPS following ventric- syndrome. Am J Cardiol 1961; 7: 73-82.

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3. Engle MA, Gay WA, Kaminsky ME, et al. 251-7. The postcardiotomy syndrome then and now. 8. Clapp SK, Garson A, Gutgesell HP, Cooley Curr Probl Cardiol 1978; 3: 5-40. DA, McNamara DG. Postoperative pericar- 4. Kennedy HL, Das SK. Postmyocardial in- dial effusion and its relation to postpericar- farction (Dressler's) syndrome; report of a diotomy syndrome. Pediatrics 1980; 66: 585- case with immunological and viral studies. 8. Am Heart J 1976; 91: 233-9. 9. Heine WI, Friedman H, Mandell M, Gold- 5. Tabatznik B, Issacs JP. Postpericardiotomy berg H. Antibodies to cardiac tissue in acute syndrome following traumatic hemopericar- ischemic heart disease. Am J Cardiol 1966; dium. Am J Cardiol 1961; 7: 83-96. 17: 798-803. 6. Engle MA, Zabriskie JB, Senterfit LB, Gay 10. Liem KL, ten Veen JH, Feltkamp TEW, WA Jr, O'Loughlin JE Jr, Ehlers KH. Viral Durrer RD. Incidence and significance of illness and the postpericardiotomy syndrome; heart muscle antibodies in patients with acute a prospective study in children. Circulation myocardial infarction and unstable . 1980; 62: 1151-8. Acta Med Scand 1979; 206: 473-5. 7. Roses DF, Rose MR, Rapaport FT. Febrile 11. Engle MA, Ehlers KH, O'Loughlin JE, et al. responses associated with cardiac surgery; re- The postpericardiotomy syndrome; iatro- lationships to the postpericardiotomy syn- genic illness with immunologic and virologie drome and to altered host immunologic reac- components. Cardivasc Clin 1980; 11/2: 381- tivity. J Thorac Cardiovasc Surg 1974; 67: 91.

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