|Year : 2018 | Volume
| Issue : 1 | Page : 75-77
An unusual complication of perimembranous ventricular septal defect with infective endocarditis: Vegetations obstructing right ventricular outflow tract and pulmonary valve
K Venkatesan Kongunattan, N Swaminathan, S Venkatesan
Institute of Cardiology, Madras Medical College, Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||9-Mar-2018|
Dr. K Venkatesan Kongunattan
NO-77, South Agraharam, Thenkarai, Periyakulm, Theni, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Ventricular septal defect (VSD) is usually diagnosed in childhood. In adults, it is less often diagnosed due to spontaneous closure of some VSD's during the early years of childhood. Perimembranous VSD is a defect seen in the upper part of the septum and near the valves occurring in nearly 75% of patients. Although spontaneous closure has been reported in VSD, with about 30%–40% closing before 2 years of age and the remainder of cases closing by about 4 years of age, it is rare for VSD's to close after 8 years of age. Most of the small VSD's will remain asymptomatic, but one of the long-term complications of a small VSD is the development of infective endocarditis (IE). Most of the studies in adults with small VSD's and IE had many complications such as arrhythmias, subaortic and sub-pulmonic stenosis, and exercise intolerance. Here, we report a patient with small perimembranous VSD who was asymptomatic in childhood but suddenly become symptomatic due to development of IE. In this patient, the vegetations were large, multiple chunky linear vegetations partly sealing the VSD defect and also occluding the right ventricular outflow tract and pulmonary valve mimicking severe pulmonary stenosis, which was managed conservatively.
Keywords: Infective endocarditis, right ventricular outflow tract obstruction, vegetations, ventricular septal defect
|How to cite this article:|
Kongunattan K V, Swaminathan N, Venkatesan S. An unusual complication of perimembranous ventricular septal defect with infective endocarditis: Vegetations obstructing right ventricular outflow tract and pulmonary valve. J Indian Acad Echocardiogr Cardiovasc Imaging 2018;2:75-7
|How to cite this URL:|
Kongunattan K V, Swaminathan N, Venkatesan S. An unusual complication of perimembranous ventricular septal defect with infective endocarditis: Vegetations obstructing right ventricular outflow tract and pulmonary valve. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2018 [cited 2020 Jun 7];2:75-7. Available from: http://www.jiaecho.org/text.asp?2018/2/1/75/227033
| Introduction|| |
Ventricular septal defect (VSD) is usually diagnosed in childhood. In adults, it is less often diagnosed owing to spontaneous closure of some VSD's during the early years of childhood. Perimembranous VSD is a defect seen in the upper part of the interventricular septum and near the valves occurring in nearly 75% of patients. Although spontaneous closure has been reported in VSD, with about 30%–40% closing before 2 years of age, and the remainder of cases closing by about 4 years of age, it is rare for VSD's to close after 8 years of age. Most of the small VSD's will remain asymptomatic, but one of the long-term complications of a small VSD is the development of infective endocarditis (IE). Most of the studies in adults with small VSD's and IE had many complications such as arrhythmias, subaortic and sub-pulmonic stenosis and exercise intolerance. Here, we report a patient with small perimembranous VSD who was asymptomatic in childhood, but suddenly become symptomatic due to development of IE with obstructive features.
| Clinical Presentation|| |
A 13-year-old female was admitted with the complaints of fever of 1-month duration. She was known to have small perimembranous VSD of around 3 mm in size from 4 years of age and was on regular follow-up. She was admitted, and all fever profile investigations were sent. On examination, she was mildly icteric, and an ejection systolic murmur with loud P2 was heard. Investigations showed hemoglobin of 9 g %, total leukocyte count of 14,000 cells/cubic mm, blood sugar and urea within the normal limits and normal electrocardiogram [Figure 1]. Surprisingly, her echocardiogram showed large, chunky, multiple linear vegetations attached to the right ventricular outflow tract (RVOT) side closing the VSD defect [Figure 2]. In addition, multiple linear vegetations were seen going into the main pulmonary artery during systole and coming into RVOT during diastole producing pulmonary valve obstruction (pseudoobstruction) with a peak gradient of 117 mmHg [Figure 3] and [Figure 4]. Ultrasound of abdomen was normal, but her liver function tests showed raised liver enzymes, with bilirubin of 3.5 mg/dl, blood culture showed no growth in all the three samples, and microscopic slide agglutination test was positive. Since the patient had fever with jaundice, we also thought of leptospirosis and was started on treatment. She received antibiotics for 3 weeks but continued to have fever spikes, and the vegetations increased in size and extended into the surrounding area involving the anterior mitral leaflet. Hence, we changed the antibiotics to parenteral piperacillin, amikacin, and oral doxycycline for 4 weeks. The patient was symptomatically better, and no fever spikes were seen. Repeat echocardiogram showed the size of vegetations had significantly decreased and the pulmonary valve gradient also decreased with a peak gradient of 48 mmHg. She was discharged and was on regular follow-up.
|Figure 2: Vegetations seen in the right ventricular outflow tract crossing and obstructing the pulmonary valve flow (parasternal short axis and subcostal views)|
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|Figure 4: Images showing gradient across the ventricular septal defect and pulmonary valve|
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| Discussion|| |
VSD is a benign cardiac lesion the prognosis of which can be severely compromised with the development of IE. Surgical repair of VSD reduces the risk, but does not entirely exclude it due to the development of some complications later on. Right-sided endocarditis is mainly a disease of intravenous drug abusers, but can also occur in nondrug addicts. The incidence of right-sided IE ranges from 5% to 10%. The majority of cases involve the tricuspid valve, while isolated pulmonary valve endocarditis is rare. It is assumed that its incidence is low due to the low-pressure gradients within the right heart, the lower prevalence of congenital malformations, the lower oxygen content of venous blood, and the differences in the vascularization of the right heart endothelium. Most cases of pulmonary valve endocarditis in children are secondary to the presence of a congenitally abnormal pulmonary valve and in adults secondary to intravenous drug abuse. Necropsy studies of patients with right-sided IE and of drug addicts have shown that vegetations were ten times more common in the tricuspid valve than in the pulmonary valve. The involved valves were nearly always anatomically normal before the onset of the cardiac infection. The survival of patients with congenital heart disease has increased in number and age of the population susceptible to IE has also increased.
The patients most susceptible to IE are those with tetralogy of Fallot, patent ductus arteriosus (PDA) and VSD's. Congenital and acquired valve deformities play an important role in the pathogenesis of the infection and are responsible for the increase of IE in adults. Vegetations usually develop on the low-pressure side of the defect with endocardial trauma and are usually downstream from the site of the lesions. In patients with PDA, vegetations first develop at the pulmonary end of the ductus, while in VSD, vegetations are usually seen on the right ventricular margins of the defect. Patients with subcristal VSD and aortic regurgitation have a particularly high incidence of IE involving the prolapsed cusps of the aortic valve. In patients with subpulmonary VSD, the right coronary sinus (RCS) bulges into the RVOT because of the abnormal position of the defect and the jet stream passing through the defect. The herniated RCS may cause a mild-to-moderate pressure gradient across the RVOT while a prolapsed right coronary cusp (RCC) may cause aortic regurgitation.
Although the infection occurs on the “jet lesion” with a traumatized endocardium on the right ventricular side, in patients with subpulmonary VSD, the infection may extend from the deformed RCC to the essentially normal pulmonary valve, usually located on the low-pressure side without endocardial damage. In our case, the patient responded to antibiotics and became asymptomatic within 4 weeks of appropriate antibiotics, even though echocardiography showed an only mild decrease in the size of vegetation. As she was asymptomatic and inflammatory markers were normalized, we did not consider the option of surgery and put the patient on regular follow-up. A review of published data indicated that the role of surgery in isolated pulmonic valve endocarditis is unclear. Recurrent pulmonary emboli are not an indication for surgery, which is only needed if fever persists despite 3 weeks of appropriate antibiotic treatment in the absence of a pulmonary abscess. Surgical options include debridement of the infected area, vegetation excision with either valve preservation or valve repair/replacement.
| Conclusion|| |
IE with large obstructive vegetations in the right heart due to a perimembranous VSD is an unusual presentation and requires a high degree of suspicion, particularly if the presence of congenital heart disease is unknown. Vegetations attached to the VSD are particularly difficult to diagnose. We detected large multiple linear vegetations in the RVOT due to impingement of jet from the VSD, and these vegetations were prolapsing into the pulmonary artery after crossing the pulmonary valve producing pseudoobstruction. This patient was managed with conservative treatment. We diagnosed the condition by two-dimensional echocardiography, which appears to be a more specific imaging modality and useful in treating the patient and assessing prognosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]