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a Division of Cardiovascular Surgery, Children's National Medical Center, Washington, District of Columbia
b Division of Cardiology, Children's National Medical Center, Washington, District of Columbia
c Division of Pathology, Children's National Medical Center, Washington, District of Columbia
Accepted for publication August 3, 2009.
* Address correspondence to Dr Jonas, Department of Cardiovascular Surgery, Children's National Medical Center, 111 Michigan Ave NW, Washington, DC 20010 (Email: rjonas{at}cnmc.org).
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Introduction |
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A 41-year-old woman from Kuwait, a known case of Noonan syndrome and hypertrophic cardiomyopathy, presented with chest pain, palpitations, dizziness, and decreased exercise tolerance. She had undergone resection of muscle in both ventricles, closure of an atrial septal defect, tricuspid annuloplasty, and pulmonary valvotomy 24 years earlier. Two years later, she underwent open pulmonary valvotomy and right ventricular outflow reconstruction for residual pulmonary obstruction. Six months before referral to Children's National Medical Center, atrial fibrillation and signs of congestive cardiac failure developed.
A chest roentgenogram demonstrated marked cardiomegaly. Echocardiography revealed multiple mobile masses in both ventricles, with a large mass in the left ventricular outflow tract. She also had moderate pulmonary and tricuspid regurgitation. Cardiac catheterization confirmed a pedunculated mass in the left ventricular outflow tract attached to the septum and at least two other masses in the right ventricle attached to the free wall. The coronary arteries were ectatic, with no evidence of coronary artery disease.
A cardiac magnetic resonance image (MRI) confirmed the presence of multiple intracardiac masses as well as right heart enlargement with severe pulmonary insufficiency. MRI of the brain showed a distal left carotid artery aneurysm with no evidence of infarction. There was also evidence of chronic pulmonary thromboembolism from the right-sided tumors on computed tomography of the chest, with no evidence of deep vein thrombosis. Preoperative blood typing revealed a rare (Cellano) antibody.
The patient underwent an open heart operation for excision of the intracardiac masses and pulmonary valve replacement. Transesophageal echocardiography confirmed multiple masses in both the ventricles and moderate pulmonary insufficiency. Sternotomy was performed after cardiopulmonary bypass was established electively by cannulation of the femoral vessels. A right ventricular outflow tract incision through the previous transannular patch was used to approach the masses in the right ventricle, and an aortotomy was used to approach the left-sided tumors.
Multiple lesions—35 to 40—were studded all over the endocardial surface of both the ventricles. Although most lesions appeared filamentous and anemone-like, others were raised and circumscribed. The largest lesion on the right side measured 5 to 6 mm, with most lesions smaller than 4 mm. The mass on the left was 8 to 10 mm and lay 3 cm below the aortic valve on the septum (Fig 1). There were multiple smaller masses. A combination of sharp and blunt dissection was used to excise these masses, after which the base of all tumors was cauterized. The pulmonary valve was replaced using a 21-mm Perimount (Edwards Lifesciences, Irvine, CA) bovine pericardial valve.
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Histopathologic examination of the masses from both ventricles revealed papillary fronds lined by endothelial cells with focal calcifications and thus confirmed the diagnosis of multiple PFE (Fig 2).
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PFEs are not associated with any syndromes. Although our patient also had Noonan syndrome, there is no known association between this and cardiac tumors. Noonan syndrome is associated with cardiac malformations such as pulmonary stenosis, for which the patient had earlier undergone operations. Multiple PFEs occur in less than a tenth of the cases.
Kurup and colleagues [3] suggested that multiple PFEs tend to develop after previous open heart procedures and involve nonvalvular endocardium in the chambers closest to the previous operation. Ngaage and colleagues [1] found that 19% of the PFEs had concurrent hypertrophic cardiomyopathy (HOCM). HOCM was also an associated finding in the Cleveland study of cardiac PFE [2]. The maximum number of lesions reported in any single patient to date is 8 [2, 5]. Our patient had between 35 and 40 tumors, which suggested a more aggressive nature of the tumor. Although our patient had evidence of pulmonary embolism, there was no conclusive evidence of systemic embolism.
There has been some controversy about whether PFE represents a true tumor or is a reactive tumoral lesion [1]. Grandmougin and colleagues [6] proposed a viral etiology for these tumors. Others have suggested that they are hamartomas because of their mimicry of tendinous cords, whereas some think that they are organizing thrombi, a theory supported by the presence of fibrin within the cores.
HOCM is characterized by myocyte hypertrophy and disarray along with endocardial and interstitial fibrosis. It is likely, as pointed out by Ngaage and colleagues [1], that this generalized abnormality of myocardial architecture predisposes to the development of multiple PFEs in HOCM.
Although a primary PFE can occur de novo in an otherwise normal heart, multiple lesions close to prior surgical sites also suggest an aberrant reactive response to trauma as a mechanism of development. It is likely that persistent turbulence at the operated sites leads to endothelial hyperplasia and PFE, as also suggested by Kurup and colleagues [3]. This iatrogenic mechanism is probably different from that operational in HOCM, which is an intrinsic abnormality. We speculate that a combination of such iatrogenic and intrinsic mechanisms predisposes to the development of multiple PFEs, as it did in our patient.
Most of the lesions in our case had anemone like fronds, but some were pearly white with raised edges. In their experience with multiple lesions after open heart operations, Okamoto and colleagues [7] also found that the morphology was not typical, making diagnosis by echocardiogram difficult. We believe that the varied morphology of the tumor as seen in our patient represents stages of tumor growth rather than reflecting a different etiology, and the aggressive nature of the tumor provided us an opportunity to witness the tumor in its different stages of growth.
Recurrence has not been reported after resection of multiple tumors, including those thought to be "reactive" lesions adjacent to prior surgical sites [1, 8]. Because the original stimulus to the reactive lesion is not completely eliminated by surgical excision of the tumor and correction of the heart defect, close follow-up is warranted, especially because the reactive lesions can present with atypical morphology [7].
In conclusion, our patient with numerous PFEs (35 to 40 lesions) had both HOCM and a history of multiple open heart operations, offering both as possible causes of the numerous lesions. We hypothesize that this combination of predisposing factors may have led to the development of such a locally aggressive form of multiple PFEs with varied morphology. Closer follow-up is required in such cases even though recurrence has not been reported.
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This article has been cited by other articles:
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  G. Lazaros, M. Vavuranakis, D. A. Vrachatis, and C. Stefanadis A 53-Year-Old Woman With Recurrent Transient Ischemic Attacks Chest, October 1, 2010; 138(4): 1004 - 1009. [Full Text] [PDF]
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 Y. Okamoto and M. Matsumoto Multiple Papillary Fibroelastomas Ann. Thorac. Surg., June 1, 2010; 89(6): 2072 - 2073. [Full Text] [PDF]
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