| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
Ann Thorac Surg 2001;71:2036-2038
© 2001 The Society of Thoracic Surgeons
a Apollo Hospitals, Chennai, India
Accepted for publication May 5, 2000.
Address reprint requests to Dr Oomman, Apollo Hospitals, Greams Lane, Chennai, Tamil Nadu 600006, India
e-mail: dr-oomman{at}mailcity.com
 |
Abstract |
|---|
 Top Abstract IntroductionCommentReferences |
|---|
|
Introduction |
|---|
|
TopAbstractIntroductionCommentReferences |
|---|
A 37-year-old woman was admitted to our center with New York Heart Association (NYHA) class III dyspnea of 8 months’ duration. She was apparently well until September 3, 1997, when she developed fever, headache, and breathlessness. On examination by the local physician, she was found to have bilateral crepitations and rhonchi, and was treated with ciprofloxacin. In September 1997, she had a similar episode, which once again subsided with antibiotics.
In October 1997, while walking, she had severe breathlessness and chest discomfort. She sought medical help the next day and was diagnosed as having acute myocardial infarction. She was treated conservatively and did not receive thrombolytic therapy, but her dyspnea did not subside. She continued to have NYHA class III dyspnea. Her symptoms worsened and required prolonged hospitalization in January 1998.
There was no history of hypertension, diabetes mellitus, or bronchial asthma. Clinical examination revealed a moderately built tachypneic patient with a respiratory rate of 28 per minute, pulse of 106 per minute, and blood pressure of 120/80 mm Hg. Her jugular veins were distended. The apex beat was felt in the sixth left intercostal space, 3 cm lateral to the midclavicular line. An S3 gallop rhythm and a 2/6 early systolic murmur were audible at the apex. Her routine cytological and biochemical parameters were within normal limits. An echocardiogram revealed dilated left ventricle with systolic and diastolic bulge suggestive of aneurysm of the apical septal and anterolateral segments. The rest of the anterior wall, lateral wall, and septum were hypokinetic. The ejection fraction was 34%. There was mild mitral regurgitation, explaining the clinically audible murmur.
Her cardiac failure was controlled with digoxin, diuretics, angiotensin-converting enzyme (ACE) blockers, and nitrates. Cardiac catheterization revealed a grossly dilated left ventricle (LV) with a large aneurysm of the anterolateral region with severe LV dysfunction. Pressure data were as follows (mm Hg): right artery mean: 23; right ventricle: 48 (systolic), 24 (diastolic); pulmonary artery: 48 (systolic), 27 (diastolic), mean 33; pulmonary artery wedge (PAW) mean: 24; LV: 130/34; aorta: 120/70, mean 90.
The coronary arteries were normal, except proximal left anterior descending artery (LAD) irregularity, which in view of her clinical symptoms and electrocardiographic changes raised the suspicion of a fully recanalized LAD in spite of the absence of thrombolytic therapy.
She was diagnosed as having an LV aneurysm due to an anterior wall myocardial infarction, on the basis of symptoms, electrocardiogram changes, and LV aneurysm in LAD territory with a suspicion of fully recanalized LAD on coronary angiogram.
She was taken up for aneurysmectomy, and Dor’s endoaneurysmorrhaphy was performed. The operation was performed via midline sternotomy and standard cardiopulmonary bypass technique. Body temperature was reduced to 32°C. The myocardial protection was by fibrillated but perfused heart. Visual examination of the heart showed whitish, opalescent, aneurysmal anterior and anterolateral wall of LV. The LV was opened lateral to the LAD. Endoaneurysmorrhaphy was performed as described by Dor [1] using Fontan suture and Dacron patch graft. The edges of the LV were closed over the patch with Prolene. Peroperative transesophageal echocardiogram showed no leak across the patch and mild mitral regurgitation (MR). Her hemodynamics improved and she did not require intraaortic balloon pump support. She required adrenaline infusion for 8 hours and dopamine infusion for 24 hours. There were no significant arrhythmias in the postoperative period. There was progressive reduction in end systolic volume and improvement in ejection fraction. Her symptoms reduced and effort tolerance improved. From class IV, her symptoms came down to class II.
The histopathology showed myocardium with hypertrophied muscle fibers infiltrated with Langhan’s type cells and foreign body-type multinucleated giant cells, lymphocytes, and eosinophils. They did not tend to form discrete granulomas. Stains for acid-fast bacillus (AFB) and fungus were negative.
The features of a postinfarction aneurysm like a thin wall comprised primarily of dense collagen, with or without underlying mural thrombus, were absent. The microscopic picture was different from sarcoidosis and suggested active giant cell myocarditis with focal fibrosis.
The patient was discharged 20 days postoperatively on digoxin, diuretics, ACE blocker, and anticoagulants. The condition and prognosis was discussed with the patient and relatives, and it was decided to give her a course of prednisolone only (50 mg daily to be tapered) until review and to consider other pharmacological or transplant options if her condition deteriorated.
The patient was followed by telephone contact and correspondence. She came for review 6 and 13 months after surgery (29 months after onset of symptoms). Her symptoms had decreased considerably. She could walk 2 to 3 kilometers at a stretch and do all household activities without symptoms. Echocardiogram revealed improvement in ejection fraction from 34% to 48%. There was mild MR. The end systolic volume had reduced from 99 to 64 mL. In view of the clinical and echocardiographic improvement, it was decided not to start high-dose immunosuppressive therapy or to consider transplantation. At 13 months of surgery (2.5 years after onset of symptoms), the patient is in NYHA class II. Echocardiographic parameters were similar to that at 6-month follow-up.
|
Comment |
|---|
|
TopAbstractIntroductionCommentReferences |
|---|
The presenting symptoms in the majority are those of heart failure, which is often progressive and refractory. A minority can present with a syndrome of chest pain and electrocardiographic findings suggestive of acute myocardial infarction [3]. Rarely, complete heart block can also be a presenting feature. Almost half the patients develop refractory ventricular arrhythmias during the course of their disease.
Until recently, it was considered to be a universally fatal disease. Most patients die of congestive heart failure, although some have survived long periods, often after immunosuppressive therapy [4, 5]. The immunosuppressive agents tried include corticosteroids alone or in combination with azathioprine, cyclosporine, and OK T3. The multicenter giant cell myocarditis study group investigators found that the median survival from symptom onset with corticosteroids alone is 3.8 months, with cyclosporine combination therapy was 12.6 months, and without immunosuppression was 3 months [3]. Cardiac transplantation is frequently the option in view of the universally bad prognosis with immunosuppressants. However, the disease can recur in the transplanted heart [3, 6]. Twenty-six percent of the patients undergoing heart transplantation exhibited giant cell infiltrate in the transplanted hearts postoperatively [3].
Our patient had many unique features. The first was the presentation mimicking acute myocardial infarction. The onset was with dyspnea and chest discomfort, with electrocardiogram suggestive of acute myocardial infarction. Six percent of giant cell myocarditis can present with a syndrome of chest pain and electrocardiogram changes suggestive of myocardial infarction. But this was not suspected clinically due to its rarity. Second, the patient survived almost 12 months without definitive therapy, although she had deteriorated to NYHA class IV. The rate of death or cardiac transplantation was 89%, with a median survival of 5.5 months from the onset of symptoms to the time of death or transplantation in the largest series [3]. It is highly unlikely that a common disease like coronary artery disease coexisted with this rare disorder (giant cell myocarditis) in our patient, as the histopathological features were suggestive more of ongoing myocarditis than chronic ischemic heart disease. Third, the treatment modality was unique. Dor’s endoaneurysmorrhaphy definitely improved the quantity and quality of life of the patient. The control of cardiac failure and the improvement in LV function might have been the contributory factors, as most patients die of congestive heart failure. The lack of progression of the disease indicates that the disease was limited to the resected segment. The impact of immunosuppression, if any, would have been insignificant, as the patient was given low-dose corticosteroids only, which in isolation is associated with a median survival of 3.8 months only.
|
References |
|---|
|
TopAbstractIntroductionCommentReferences |
|---|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |
