Tumor Plop in a Febrile Child: Unusual Presentation of Tricuspid Valve Endocarditis.

Tricuspid valve endocarditis in a structurally normal heart of child is extremely rare and mysterious disease, which may mimic several other illnesses. We are reporting here a six-year-old child who presented with prolonged fever and unusual auscultatory sound of tumor plop, later proven to having tricuspid valve endocarditis by 2D echocardiography. The patient underwent surgical excision of the vegetations with repair of the tricuspid valve leaflets with successful outcome.

CASE REPORT

A 6-year-old male child presented in the emergency with high grade intermittent fever and erythematous rashes all over the face along with loss of appetite and weight for two weeks. Before being admitted in our hospital, he had received oral drugs prescribed on the line of lower respiratory tract infection. There was no history of peripheral intravenous cannulation, instrumentation, and blood transfusion. On arrival, he was conscious, febrile, dyspneic, pale, irritable with generalized lymphadenopathy, mild abdominal distension, and pedal edema. His temperature was 104°F, pulse was 140 per min, regular, blood pressure was 100/60 mm Hg and respiratory rate was 30 per min. At the admission, examination of cardiovascular system revealed no significant finding. Lung examination was suggestive of left side collapse consolidation-pneumonic. He was also having 4-cm soft, non-tender hepatomegaly. Neurological examination including fundus evaluation was normal. A working diagnosis of prolonged pyrexia due to left-sided pneumonia with anemia and congestive cardiac failure was made, and the possibilities of typhoid fever and malaria were also entertained. Blood, urine, and sputum cultures were taken. Empirical antibiotic therapy and supportive therapy were started along with diuretics.

Blood investigations revealed parameters suggestive of infective pathology. Peripheral smear showed normocytic hypochronic anemia with neurophilia and moderate thrombocytopenia and mild shift to left pattern. Malarial parasites were absent in smear. Widal test was also found to be negative. Urine microscopy was normal and urine cultures were sterile. ECG was within normal limits and chest X-ray showed left lower lobe pneumonic patch with left sided mild pleural effusion. Spikes of 103°F continued despite of intravenous antibiotics. On 2nd day, repeat thorough examination of cardiovascular system revealed faint systolic murmur with a tumor plop. Blood culture yielded growth of Staphylococcus aureus and antibiotics were changed according to sensitivity. Due to auscultatory tumor plop finding, transthoracic 2D echocardiography was done, and it showed large echodense heterogeneous mass (suspected vegetation) attached to tricuspid valve apparatus [Figures 1 and 2], mild to moderate tricuspid regurgitation (TR) with moderate pulmonary artery hypertension (PAP, 45 mm/Hg) [Video 1]. Echocardiography revealed no predisposing condition in heart or pre-existing abnormalities of the any cardiac valve.

Figure 1

Apical four-chamber view showing large vegetation attached with the tricuspid valve leaflet

Figure 2

Apical four-chamber view showing large vegetation attached with the tricuspid valve leaflet

Repeat two blood cultures sent on 2nd hospital day also grew S. aureus. After due consent of the parents, the child was taken up for surgery. Midline sternotomy was done and under cardiopulmonary bypass. The right atrium was opened and the tricuspid valve was examined. Two vegetations were found attached to anterior leaflet measuring 2.5 × 2.0 sq.cm and posterior leaflet measuring 2.0 × 1.5 sq.cm [Figure 3]. Both masses were excised followed by reconstruction of posterior leaflet taking care to preserve the native valve. Modified DeVega annuloplasty done and competency of tricuspid valve was examined. The patient was weaned off from cardio pulmonary bypass gradually. Later, there was complete clinical symptomatic resolution with smooth postoperative recovery. We treated our patient with ceftriaxone/Cefixime for 6 weeks. Postoperative 2D echocardiogram revealed trivial tricuspid regurgitation, no visible mass or vegetation in the right atrium. Histopathological report shows acute fibrinous inflammation with focal suppuration and many bacterial colonies confirming of infective endocarditis (IE) in the tricuspid valve.

Figure 3

Intraoperative photograph showing opened Right atrium and vegetation attached with the Tricuspid valve anterior leaflet

DISCUSSION

Childhood IE was previously only a rare complication of rheumatic heart disease. However, now, IE in childhood is increasingly common and difficult surgical problem in cardiovascular units but reports describing surgical experiences with childhood IE, specially of tricuspid valve are very less.[12]

Tricuspid valve endocarditis (TVE) in children with structurally normal heart is very rare diagnosis. There are only a few case reports till now. In TVE, systemic presentation and extracardiac complications generally predominate the clinical picture and hide the underlying main valvular pathology.[3] The patients may present with fever, respiratory symptoms, flu-like illness, or diarrhea as our patient presented. He did not have any history of central venous line cannulation or immunocompromised state. In our case, the respiratory infection with anemia was recognized initially, but the main cause endocarditis was missed. This pneumonia usually arises due to migration of septic emboli from vegetations over tricuspid valve into pulmonary arteries. The consequent delay in correct evaluation may delay appropriate therapy and decrease the probability of a successful outcome.[4]

The clinical presentation usually consists of persistent fever associated with pulmonary manifestations, anemia, and microscopic hematuria, signs that constitute the “Tricuspid syndrome”. TVE does not usually result in any detectable murmur, which complicates the diagnosis. The absence of peripheral stigmata of endocarditis or relevant murmurs in the majority of cases is worth mentioning. Right-sided endocarditis involving the tricuspid valve should be included in the differential diagnosis of patients with staphylococcal or streptococcal bacteremia accompanied by clinical and or radiological signs of pulmonary involvement.[56]

Our patient had unusual tumor plop sound, which suggested us to go ahead with early 2D echocardiography. In this case, large vegetation produced a diastolic sound that is found as “tumor plop”. This sound arises from obstruction to ventricular in-flow that occurs because the vegetation/mass comes to rest over the tricuspid annulus and functionally equivalent to tricuspid valve stenosis and also due to damage to the tricuspid valve a concurrent regurgitant murmur was produced. Even in the absence of classic predisposing risk factors for right-sided endocarditis, a diagnosis of right-sided endocarditis should be considered and echocardiography should be promptly carried out to document the presence of vegetation. It can detect valvular vegetation in 80-100% of patients with endocarditis.[7]

Duke or modified Duke Criteria may be used to make the diagnosis in otherwise unclear cases of endocarditis. The suggested major criteria were vegetations demonstrable on echocardiography associated with fever. The minor criteria comprised of multiple positive blood cultures, evidence of septic pulmonary emboli, absence of systemic emboli, and a murmur compatible with tricuspid involvement. Either two major or one major and three minor criteria make the diagnosis of TVE.[8] In cases complicated by sepsis, severe valvular dysfunction, conduction disturbances, or embolic events, empirical antimicrobial therapy should be started after three blood cultures have been taken.[9]

In right-sided IE, surgery is indicated for large (>10 mm) or increasing vegetation size, severe heart failure, fungal endocarditis, annular or aortic abscess, evidence of valvular dysfunction, persistent infection after a prolonged period (7-10 days) of appropriate antibiotics, as indicated by presence of fever, leukocytosis, and bacteremia-assuming that there are no non-cardiac causes for infection and recurrent emboli after appropriate antibiotic therapy. In our case, we decided to go for surgery in view of persistent sepsis and large vegetation size. If untreated, IE is a fatal disease. Major diagnostic (first of all echocardiography) and therapeutic progress (mainly surgery during active IE) have contributed to some prognostic improvement during the last decades. If the diagnosis is delayed or appropriate therapeutic measures postponed, mortality is still high.[10]

Through our report, we strongly emphasize that one should have high clinical suspicion in febrile pediatric cases and should go for early 2D echocardiogram to detect infective endocarditis. Few studies have also suggested that any child with acute Staphylococcal sepsis should be subjected to 2D echo particularly if fever does not subside despite antibiotic therapy. The underlying diagnosis could have been missed if 2D echo was not used. We should aim at surgical intervention before the onset of systemic embolization and irreversible damage to the valvular apparatus.[10]