|Year : 2017 | Volume
| Issue : 2 | Page : 100-102
Left ventricular diverticulum with ventricular premature complexes in fetal and postnatal period: A rare presentation
Niraj Kumar Dipak1, Sumitra Venkatesh2, Punya Pratap Kujur3, Shakuntala Sanjay Prabhu2
1 Department of Neonatology, B. J. Wadia Hospital for Children, Nowrosjee Wadia Maternity Hospital, Mumbai, Maharashtra, India
2 Department of Pediatric Cardiology, B. J. Wadia Hospital for Children, Nowrosjee Wadia Maternity Hospital, Mumbai, Maharashtra, India
3 Department of Pediatrics, B. J. Wadia Hospital for Children, Nowrosjee Wadia Maternity Hospital, Mumbai, Maharashtra, India
|Date of Web Publication||13-Apr-2017|
Niraj Kumar Dipak
Department of Neonatology, B. J. Wadia Hospital for Children, Nowrosjee Wadia Maternity Hospital, Acharya Donde Marg, Parel, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
Congenital ventricular diverticulum is a rare congenital anomaly, which is defined as an outpouching structure that contains all the three layers of heart; that is, endocardium, myocardium, and pericardium and shows synchronous contractility. It remains clinically asymptomatic. The treatment of the left ventricular diverticulum (LVD) is undefined. We report a rare case of LVD presented as fetal bradycardia and further postnatally as ventricular premature complexes. A conservative approach with follow up was adopted in the case.
Keywords: Congenital left ventricular diverticulum, left ventricular aneurysm, left ventricular outpouching, ventricular premature complexes, transaxial helical computed tomography cardiac angiogram
|How to cite this article:|
Dipak NK, Venkatesh S, Kujur PP, Prabhu SS. Left ventricular diverticulum with ventricular premature complexes in fetal and postnatal period: A rare presentation. J Clin Neonatol 2017;6:100-2
|How to cite this URL:|
Dipak NK, Venkatesh S, Kujur PP, Prabhu SS. Left ventricular diverticulum with ventricular premature complexes in fetal and postnatal period: A rare presentation. J Clin Neonatol [serial online] 2017 [cited 2019 Sep 21];6:100-2. Available from: http://www.jcnonweb.com/text.asp?2017/6/2/100/204514
| Introduction|| |
Left ventricular diverticulum (LVD) comes up for discussion when an outpouching of the left ventricle (LV) is detected during prenatal sonography. Various ways to characterize further are their location: apical or nonapical; myocardial layers: muscular or fibrous; contractility: synchronous or paradoxical. Apical diverticula are always associated with midline thoracoabdominal defects (epigastric pulsating diverticulum or large omphalocele) and other structural malformations of heart. Nonapical diverticula are usually isolated defects. Most patients with LVD remain clinically asymptomatic and further, no complication are observed during long-term follow-up. However, LVD can potentially give increase to ventricular tachyarrhythmia, systemic embolism, sudden death, spontaneous rupture, and severe valvular regurgitation. The treatment of asymptomatic and isolated LVD is still undefined. We report a rare presentation of LVD presenting with fetal bradycardia and ventricular premature complexes (VPCs) after birth.
| Case Report|| |
A 33-year-old primigravidae was referred to our center at 265/7 weeks for the detection of LV outpouching of 13 mm × 10 mm size with persistent ectopic beats with fetal bradycardia. There was no associated polyhydramnios, oligohydramnios, or hydrops fetalis. Pregnancy was of spontaneous conception and nonconsaguinous. Mother was positive for autoantibodies: anti SSA (RO) IgG 168.67 (<20) RU/ml (serum enzyme immunoassay [EIA] method); anti SSB (La) IgG 1.45(<20) RU/ml (serum EIA); ANA speckled 1:160 titer. This rhythm abnormality persisted without any fetal decompensation during the subsequent 3 sonographies. Lowest fetal heart rate noted was 62 beats/min. A male baby with birth weight 2.350 kg, head circumference 33 cm, length 46 cm (all lying between 10% and 75%), was delivered by elective LSCS, at 355/7 weeks of gestation and noted to have bradycardia at birth and transferred to neonatal Intensive Care Unit (NICU). Apart from heart rate, which varied between 66 and 86/min, he maintained vitals within normal range. 12 Lead ECG showed sinus rhythm with ventricular bigeminy [Figure 1]. Two-dimensional (2D) ECHO revealed outpouching of LV posterior wall below mitral valve, with blood flow noted through it and normal LV function [Figure 2]. Baby's Anti SSA (RO) IgG was 117.48 (<20) RU/ml (serum EIA); whereas, Anti SSB (La) IgG was 0.68 (<20) RU/ml (serum EIA). He was managed on tab metoprolol 1 mg/kg/day. After 6 days of metoprolol therapy, 12 Lead ECG were repeated, which showed decreased episodes of VPCs; that is, 3–4 normal QRS complexes followed by 1 premature complex. 64 slice transaxial helical computed tomography (CT) cardiac angiogram using 80 ml nonionic IV contrast media done after priming with metoprolol, confirmed LVD from anterolateral wall (perivalvular) with width of neck of diverticulum being 11 mm × 10 mm and approximate size of diverticulum being 17 mm × 12 mm × 13 mm [Figure 2]. On Three-dimensional CINE MODE, synchronous contraction was noted with rest of LV myocardial wall suggestive of muscular variety of LVD [Figure 2]. Left ventricular outflow tract, mitral valve, aortic root, and atrio-ventricular region were normal. For the further NICU stay of 7 days, he remained hemodynamically stable before being discharged with an outpatient follow up. Currently, he is 7 months old with adequate growth and development with no evidence of hemodynamic decompensation. There is neither an increase in size of LVD nor any problem with LV function or formation of thrombus is observed in serial echocardiography since birth.
|Figure 2: Two-dimensional ECHO, computed tomography ANGIO, magnetic resonance imaging showing left ventricular outpouching|
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| Discussion|| |
The Three main differential diagnoses are diverticulum, aneurysm, and pseudoaneurysm, if a left ventricular outpouching is detected. LVD is a rare congenital anomaly, which has been reported in 0.4% of autopsies after cardiac death and in 0.26% of nonselected patients who underwent cardiac catheterization. LVD is defined as an outpouching structure that contains all the three layers of heart; that is, endocardium, myocardium, and pericardium and displays synchronous contraction (due to presence of muscular fibers) with rest of the myocardium. Whereas, LV aneurysms, and LV peudoanerusm have fibrous walls and exhibit dyskinetic or akinetic contraction. LVD may be divided into: either congenital or acquired; apical or nonapical; muscular or fiborous. They are considered to be congenital if there is no history of conditions that have injured the myocardium and during altered embryogenesis in the first 2–3 weeks, a focal defect of mucular wall have given way to an outpouching. Other potential inciting factors are in utero viral infection, muscle and connective-tissue defects, and excessive primordial-cell stimulation. Fetal echocardiography helps to support a congenital etiology when the diagnosis is made during the prenatal period. Apical congenital ventricular diverticulum (CVD) is distinguished from nonapical CVD by the size of connection from the parent structure. Apical CVD is a fingerlike contractile pouch with narrow connection to the ventricle and associated with intra- or extra-cardiac malformations, whereas, nonapical CVD, like in the index case, is a large contractile pouch with wide connection to the ventricle and is an isolated anomaly. Most of diverticulum are apical. Nonapical diverticula arise from the anterior free wall, or left ventricular outflow tract and rarely, from both ventricles., Our index case had nonapical diverticulum arising from anteriorlateral free wall. Apical CVD is described as a part of a syndrome of midline thoracoabdominal defect (epigastric pulsating diverticulum or large omphalocele) with other intracardiac malformations (tetralogy of fallot, ventricular septal defect, tricuspid atresia, dextrocardia) that had been described by Cantrell et al. whereas nonapical CVD and Congenital ventricular aneurysm (CVA) were shown as isolated cardiac defects.
Fetal 2D ECHO and postnatal ECHO remain the main-stay of diagnosis. Multidetector CT, can evaluate the anatomy of the LV and shows the myocardial contraction in the wall of the outpouching and other cardiac structures besides the coronary arteries pathologies such as stenosis, hypoplasia, and localized intimal proliferation. Cardiac magnetic resonance imaging further characterizes the contractility, signal characteristics of LV wall, presence of fibrous tissue and necrosis in the wall of outpouchings.
Nonapical LVD remains clinically asymptomatic. In exceptional circumstances, it is fraught with complications such as gradual enlargement, thrombosis, embolism, rupture, congestive heart failure, ventricular arrhythmias, and valvular abnormalities. Because of its rarity, the true incidence of these complications is not known. In the present case, sinus rhythm with bigeminal PVCs was detected on the 1st day after birth. Every other sinus P wave was most likely hidden in the PVCs, and not conducted due to the refractory state of the ventricles after the PVCs. Perhaps that was the reason for ECG strip demonstrating heart rate of 116, whereas pulse oximeter detected bradycardia with heart rate of 50–60 [Figure 3]. Two things could explain this rhythm abnormality: (i) presence of anti-SSA antibodies and LVD was an innocent by-stander in that case, (ii) LVD was acting as an ectopic focus of activity or a site for reentry. Anti-SSA antibodies are classically associated with complete heart blocks rather than VPCs. Maternal antibodies causing fetal and neonatal VPCs are not described in literature.
|Figure 3: Pulse oximetry and electrocardiogram showing discrepancy in heart rate|
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In a symptomatic patient with LVD or when diverticulum is associated with other cardiac anomalies, surgical treatment is generally resorted to. The treatment in isolated, asymptomatic LVD is largely undefined. Some authors have suggested surgical resection for all the cases keeping potential complications such as embolism, arrhythmias and spontaneous rupture in view, whereas those pitching for conservative management are concerned with postoperative risk of resection of diverticular wall, consisting of normally contracting myocardium. Apart from VPCs, which proved to be benign, our case behaved as an asymptomatic patient with isolated LVD. As he did not show any signs of decompensation till the time of writing this article, surgical correction was not advised. Kawata et al. reported surgical resection of isolated LVD under cardiopulmonary bypass in an 9-day-old infant and concluded that surgical repair usually is not required in infancy even though it combines with other anomalies.
The authors would like to thank MD, Dr. Amdekar and MS, Dr. Aswini, BJ Wadia hospital for children, Mumbai for permitting them to publish the manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]