Isolated Cardiac Aspergillosis

Ruma Ray, ZN Singh, HS Wasir, P Chopra
Departments of Pathology and Cardiology,
All India Institute of Medical Sciences, New Delhi.

A 40-year-old man, a known case of Wolff–Parkinson–White syndrome, was admitted to the hospital in an  unconscious state. In spite of medical treatment, the patient died within two hours of admission. At autopsy, the  deceased was found to have aspergillosis involving the interatrial septum, aortic valve and root of the aorta. The  rest of the organs were unremarkable. The patient did not show any obvious signs of being immunocompromised.  We report this case of isolated cardiac aspergillosis in an apparently healthy individual. (Indian Heart J 2001;  53: 505–507) 

Key Words: Cardiac aspergillosis, Wolff–Parkinson–White syndrome, Infection    

Invasive aspergillosis commonly occurs in  immunocompromised individuals, the lung being the  commonest site involved.1,2 Isolated involvement of extra-pulmonary  organs such as the heart has rarely been  documented in the literature.2,3 We report a rare case of  isolated cardiac aspergillosis involving the interatrial  septum, aortic valve and root of the aorta in an ‘apparently  healthy’ individual, where the diagnosis could be made only  on autopsy. 

Case Report 

This 40-year-old male was admitted in an unconscious state  to the cardiology department with complaints of dyspnea  and palpitation for the past three days. The patient was  diagnosed at another hospital as a case of Wolff–Parkinson–  White (WPW) syndrome two years earlier for which he was  treated intermittently. Previous records were not available.  On clinical examination, the patient was found to be  cyanosed, dyspneic with a pulse rate of 240 beats/minute.  His blood pressure was 90/60 mmHg and the jugular  venous pressure was raised. Cardiac auscultation revealed  marked tachycardia and chest auscultation showed signs  of pulmonary edema. There was a nontender hepatomegaly  3 cm below the costal margin. The electrocardiogram  revealed a heart rate of 250 per minute, a broad QRS  complex and left axis deviation. Echocardiography revealed  an enlargement of both the ventricular chambers, moderate  aortic regurgitation with pulmonary arterial hypertension.  The left ventricular systolic function was found to be  reduced. 

The patient was diagnosed clinically as a case of WPW  syndrome with atrial flutter. He was treated with direct  current cardioversion, and injections of dopamine, heparin  and furosemide. In spite of the medical treatment,  terminally the patient developed bradycardia and died of  cardiac arrest within two hours of admission to the hospital.  A thoracoabdominal autopsy was performed. 

Autopsy findings: The heart weighed 350 g and was  unremarkable externally, with no evidence of a pericardial  effusion. On opening the right atrium, the smooth part of  the septum, including the fossa ovalis, showed an elevated  gray-white plaque-like lesion falling short of the tricuspid  annulus. The lesion encroached into the superior vena caval  orifice which was thus compromised. The interatrial septum  measured 2.5 cm and was hard and thickened (Fig. 1).  Grossly, the lesion mimicked a tumour and involved the  anatomical sites for the sinoatrial (SA) and atrioventricular  (AV) nodes. The right ventricle and pulmonary artery were  unremarkable. The left atrium revealed a similar raised  plaque-like area over the interatrial septum corresponding  to the lesion on the right side. The mitral valve was  unremarkable, but the left ventricle was mildly dilated. The  aortic valve cusps showed the presence of friable, red, angry-looking  vegetations measuring 3–4 cm. The root of the  aorta revealed the presence of friable hemorrhagic clots  loosely adherent to the surface. Multiple sections were  examined from both the atria including the interatrial  septum, conduction system, ventricles, aortic valve and root  of the aorta. Sections from the interatrial septum and the plaque-like area in the right atrium showed widespread  involvement of the myocardium with areas of necrosis and  foreign body giant cell reaction (Fig. 2). There were  numerous septate fungal profiles with parallel walls and  acute angle branching which were better elicited by  periodic-acid Schiff (Fig. 3) and Grocott silver methenamine  stains. Many giant cells showed the presence of fungal  fragments within their cytoplasm. A section from the aortic  vegetation revealed similar fungal profiles within a fibrin  meshwork. There was a contiguous spread to the root of  the ascending aorta. Sections taken from the areas of the  SA node, AV node and internodal region showed widespread  replacement by the fungal granuloma. The diagnosis of  aspergillosis of the interatrial septum, aspergillus  endocarditis of the aortic valve and aspergillus aortitis of  the root of the aorta was made. Meticulous gross and  microscopic examination of the other organs failed to reveal  any focus of fungal infection. The lungs had bilateral  bronchopneumonia and the left kidney had a small area of  infarction. Microscopic evaluation including special stains  for fungi, done on lung and kidney sections, did not show  the presence of fungus. 

Discussion 

Isolated cardiac aspergillosis is rare. Fisher et al.2 in a series  of 91 cases of invasive aspergillosis, have documented a  single case of a 78-year-old woman with sarcoma who  developed nodal tachycardia and myocardial infarction  several days prior to death. At autopsy, there was an  aspergillus abscess in the interventricular septum. The  disease has also been reported in a 15-year-old girl following  an allogenic bone marrow transplantation.3 Both these  cases had underlying risk factors which could be attributed  to the occurrence of aspergillosis. In fact, invasive  aspergillosis is known to occur in patients with a debilitating  disease, on steroid therapy, cytotoxic drugs and antibiotics,  or undergoing radiation therapy.2,4 Our case is unique as  the patient did not have any such recognizable predisposing  factors, and the development of invasive aspergillosis in an otherwise healthy individual is extremely rare.5 An  assessment of the neutrophil function in such a case  may bring out subtle abnormalities of phagocytosis and  impaired bactericidal activity which may contribute to a  relatively immunodeficient state.5 In the present case,  such investigations could not be carried out as the  patient succumbed to the disease following emergency  admission. 

Though our patient was diagnosed as a case of WPW  syndrome 2 years earlier, it is difficult to relate this clinical  diagnosis with the autopsy finding of cardiac aspergillosis.  Sections from the nodes and internodal areas showed the  presence of fungal granuloma without any recognizable  remnant of conduction tissue. It is possible that the patient  initially had a cardiac arrhythmic disorder compatible with  the WPW syndrome and a superimposed fungal infection  was acquired later, before his demise. 

The portal of entry for the fungus in the present case  remains unknown. The possible sources include the lungs  and the gastrointestinal tract. The lungs are the usual portal  of entry for aspergillus, but in our patient there was no  pulmonary involvement, though there was focal  bronchopneumonia. However, multiple sections examined  from these foci did not show any fungal profile.  

Our patient also had aspergillus endocarditis involving  the aortic valve, and this, together with the root of the aorta,  showed destruction with numerous fungal profiles  embedded in a fibrin-rich background. There was a paucity  of inflammatory infiltrate within these vegetations, and a  granulomatous reaction was conspicuous by its absence. It  can be speculated, therefore, that the infective vegetations  embolized in the intramyocardial artery and the organisms  were lodged in the interatrial septum, evoking the  subsequent granulomatous response. Aspergillus  endocarditis is thought to be an opportunistic infection and  can be seen in patients with a history of valvular cardiac surgery.6 The endocardial vegetations of aspergillus mural  endocarditis are usually contiguous with the underlying  myocardial infection.7 The mural endocardium overlying  the interatrial septal lesion in the present case was  unaffected, though the aortic valvular endocardium was  involved by a noncontiguous spread.  

The ante mortem diagnosis of cardiac aspergillosis is  difficult to reach especially in a case like the present one,  where the patient did not have any of the conventional  predisposing factors. The diagnosis is often made at  autopsy.2,6 It is possible that the infective fungal profiles are  too large to traverse the systemic capillary bed and hence  may not even enter the venous system. In such a case,  fungemia is likely to be missed even if the venous blood is  cultured. However, one needs to consider the diagnosis in a  febrile, immunocompromised patient with unexplained  cardiopulmonary decompensation, especially when the  fungus is isolated or suspected to be the cause of infection  elsewhere.3 Very rarely, the disease can primarily affect the  heart in apparently immunocompetent individuals, thereby  posing a diagnostic difficulty, as seen in our case.

Correspondence:
Dr Ruma Ray, 
Assistant Professor of Pathology, 
All India  Institute of Medical Sciences, 
New Delhi 

References 

  1. Rinaldi MG. Invasive aspergillosis. Rev Infect Dis 1983; 5: 1061–1077 

  2. Fisher BD, Armstrong D, Yu B, Gold JW. Invasive aspergillosis. Progress  in early diagnosis and treatment. Am J Med 1981; 71: 571–577 

  3. Johnson RB, Wing EJ, Miller TR, Rosenfeld CS. Isolated cardiac  aspergillosis after bone marrow transplantation. Arch Intern Med  1987; 147: 1942–1943 

  4. Williams AH. Aspergillus myocarditis. Am J Clin Pathol 1974; 61:  247–256 

  5. Ascah KJ, Hyland RH, Hutcheon MA, Urbanski SJ, Pruzanski W, St  Louis EL, et al. Invasive aspergillosis in a healthy patient. Can Med  Assoc J 1984; 13: 332–335 

  6. Kammer RB, Utz JP. Aspergillus species endocarditis. The new face of  a not so rare disease. Am J Med 1974; 56: 506–521 

  7. Walsh TJ, Hutchins GM. Aspergillus mural endocarditis. Am J Clin  Pathol 1979; 71: 640–644