Fungal infections of the heart have become more prevalent, and are being diagnosed with increasing frequency. The most common infective organism is Candida albicans, followed by Aspergillus fumigatus, and Cryptococcus. Cardiac involvement is usually associated with endocarditis, myocarditis, pericarditis, or presence of an intracardiac fungal mass. Early diagnosis is imperative, as these patients have a poor prognosis once there is cardiac involvement.Reference Alam, Higgins, Alam, Janakiraman and Gorman1
Case report
We report a two-month old female who was admitted to our hospital with the diagnosis of a cardiac mass. She was born through Caesarean section because of fetal distress, and admitted to the neonatal intensive care unit because of meconium aspiration, mild respiratory distress, and two periods of generalized, albeit short term, seizures. During this period, and umbilical venous catheter was in place for one week.
Physical examination revealed pallor, a systolic murmur graded at one to two out of six, and head circumference that was small for age. The neonate was taking regular breast feeds, and had not suffered any episode of fever.
Primary laboratory tests displayed a level of haemoglobin in the serum of 6.5 grams per decilitre, a white blood cell count of 13,500 per cubic millimetre, with 60% neutrophils on differentiation, and a platelet count of 37,000 per cubic millimetre. Acute phase reactant tests were within normal limits. Blood cultures for bacteria were negative, but Candida albicans, with a high colony count, was reported in one specimen. Immunologic evaluation failed to reveal any evidence of immune deficiency.
On the chest radiogram, the cardiothoracic ratio was seen to be slightly increased, but the 12 lead electrocardiogram was normal. Thorough echocardiographic evaluation failed to reveal any congenital cardiac anomaly, but demonstrated a round, slightly coarse, and pedunculated mass, with sharp borders, in the right atrium, arising from the septum, and having a diameter of approximately 2.3 centimetres. The mass moved in and out of the tricuspid valvare orifice, producing temporary obstruction (Fig. 1), and moderate tricuspid regurgitation.
Figure 1. The large right atrial mass protrudes through the tricuspid valve, producing temporary obstruction. Abbreviations: RA: right atrium, RV: right ventricle.
Because of urgency of the situation, the patient was referred for open heart surgery, and the mass was excised in uneventful fashion. Inspection in the operating room confirmed the presence of a pedunculated mass located in right atrium. The mass arose from the atrial septum immediately beneath the oval fossa, and attachments to the tricuspid valve raised the suspicion that the mass might be malignant.
The pathologic report revealed that the specimen was solid, nonhaemorrhagic, and creamy brown on cutting. Microscopically, there was fibrin exudation, as well as acute and chronic inflammation in an extensively necrotic background containing fungal forms, without any evidence of neoplastic tissue (Fig. 2).
Figure 2. Pathological investigation revealed acute and chronic inflammation in a necrotic background containing pseudohyphae and yeast.
We commenced treatment with Amphotericin B immediately we received the report from the pathologist, and continued such treatment for 6 weeks, when she was discharged in good condition, and with normal echocardiographic findings.
Discussion
The most common neonatal cardiac tumors are rhabdomyomas, teratomas, and fibromas, while atrial myxomas are extremely rare.Reference Shapiro2 Our case represents an even rarer cardiac mass, namely a fungal mass, but should still be considered as a possible cause of primary cardiac masses. Such masses have been described previously as primary events in young infants and premature neonates,Reference Al Dhahri, Sandor and Duncan3, Reference Daher and Berkowitz4 but Candidal infection can also be the superimposed on pre-existing cardiac tumours.Reference Joseph, Himmelstein, Mahowald and Stullman5
Prenatal and postnatal factors, such as hypoxia, haemodynamic and coagulation disturbances, are known to contribute to the formation of the vegetations.Reference Heydarian, Werthammer and Kelly6 According to one study, lesions were observed on the tricuspid valve by cross-sectional echocardiography in 5 neonates, 4 of whom presented with fetal distress.Reference Heydarian, Werthammer and Kelly6 Other reports noted that candida septicaemia, and right atrial masses, may emerge secondary to umbilical venous catheterization.Reference Daher and Berkowitz4, Reference Menahem, Robbie and Rajadurai7, Reference Johnson, Base, Thompson, Foker, Speert and Kaplan8 These reports, taken together, indicating that all such factors should be considered as the predisposing factors of Candida endocarditis.
Management depends on the frequency of complications, and progressive or regressive characteristic.Reference Shapiro2 In our case, although there was no significant cardiac manifestation, thrombocytopaenia and anaemia were present, and potential hazards, such as embolisation, obstruction, and arrhythmias, could be predicted. Because of this, we opted for surgical removal of the mass, which proved successful.
