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|Year : 2012 | Volume
| Issue : 2 | Page : 147-149
Swiss cheese ventricular septal defect with myocarditis - A rare coexistence in a neonate
AR Saboo, R Vijaykumar, S Malik, C Warke
Department of Pediatrics, Division of Neonatology, BYL Nair Ch. Hospital and TN Medical College, Mumbai, Maharashtra, India
|Date of Submission||28-May-2011|
|Date of Decision||08-Sep-2011|
|Date of Acceptance||23-Nov-2011|
|Date of Web Publication||14-Jun-2012|
A R Saboo
Department of Pediatrics, Division of Neonatology, BYL Nair Ch. Hospital and TN Medical College, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Myocarditis is defined as acute inflammation of the myocardium, usually following a non-specific flu-like illness, and encompasses a wide range of clinical presentations ranging from mild or subclinical disease to heart failure. We report a 12-day-old healthy full-term neonate who presented with abrupt onset of congestive cardiac failure (CCF) following a viral prodrome. Examination revealed persistent sinus tachycardia, lymphocytosis, gross cardiomegaly, nonspecific electrocardiogram changes with echocardiography showing Swiss cheese ventricular septal defect (VSD). VSD alone very rarely presents as early-onset cardiac failure in the absence of other precipitating factors like anemia, sepsis, hypoglycemia etc. Myocarditis, however, can mimic VSD and can present as fulminant cardiac failure in an otherwise healthy newborn. Myocarditis is usually diagnosed based on circumstantial evidence such as a recent viral infection and the sudden onset of cardiac dysfunction while ruling out other diagnostic possibilities. Elevated troponin T level is one of the most crucial noninvasive diagnostic modalities. Several trials have concluded that levels >0.055 ng/ml are statistically significant for diagnosing myocarditis in children. In our case an abrupt onset of cardiac failure following a viral prodrome and markedly elevated cardiac troponin T without sepsis and in the presence of normal coronary anatomy clinched the diagnosis of myocarditis. An early and aggressive treatment for CCF along with regular long-term follow-up plays a key role in the management of myocarditis. Role of high-dose Intravenous immunoglobulin in myocarditis has been studied by many trials with different outcomes. This is the first case report showing coexistence of VSD with myocarditis in a neonate presenting as early-onset acute cardiac failure. The report highlights the importance of screening for myocarditis in all previously normal babies presenting primarily with cardiogenic symptoms even if a structural heart disease is coexistent early in life. A simplified algorithm for work-up of CCF in a neonate is proposed.
Keywords: Congestive cardiac failure, myocarditis, neonate, Swiss cheese ventricular septal defect
|How to cite this article:|
Saboo A R, Vijaykumar R, Malik S, Warke C. Swiss cheese ventricular septal defect with myocarditis - A rare coexistence in a neonate. J Postgrad Med 2012;58:147-9
|How to cite this URL:|
Saboo A R, Vijaykumar R, Malik S, Warke C. Swiss cheese ventricular septal defect with myocarditis - A rare coexistence in a neonate. J Postgrad Med [serial online] 2012 [cited 2020 Sep 25];58:147-9. Available from: http://www.jpgmonline.com/text.asp?2012/58/2/147/97179
| :: Introduction|| |
Early-onset cardiac failure in a neonate is a rare entity. The common causes include congenital heart diseases like, patent ductus arteriosus, left-sided obstructive lesions, arrhythmias, asphyxia, genetic cardiomyopathy etc.  Myocarditis is an underdiagnosed cause of early-onset failure in neonates. Acute fulminant myocarditis has been described as an extremely severe form of myocarditis, characterized by cardiogenic shock, arrhythmias or congestive heart failure.  It is quite rare for an isolated VSD to present with cardiac failure before four to six weeks of life in the absence of associated risk factors. Till date no such case, showing coexistence of VSD and myocarditis in a neonate presenting as early-onset cardiac failure, has been reported in the literature. ,
| :: Case Report|| |
A full-term female neonate, weighing 2.7 kg, presented on Day 12, with fever, running nose and difficulty in breathing since the last two to three days. Examination revealed tachycardia (190/min) out of proportion to CCF, soft systolic murmur, respiratory distress (90/min), normal blood pressure and hepatomegaly (8 cm). Leucocytosis (26,600/cu mm) with lymphocytic predominance (67%) was present without anemia (17.6mg/dl). Chest X-ray showed gross cardiomegaly (cardio-thoracic ratio - 0.80) [Figure 1] and ECG documented sinus tachycardia, low-voltage QRS complexes and nonspecific ST-T changes. Echocardiography detected multiple muscular VSD (Swiss cheese) with the largest measuring 6 mm, good ejection fraction, normal coronary anatomy and ventricular wall motion. Investigations revealed no hypoglycemia, hypocalcemia or dyselectrolytemia. Sepsis was ruled out on the basis of the following tests;  C-reactive protein was 2.5 and 4 on Day 1 and Day 16 respectively (0-6 ng/ml), blood culture was negative on two occasions, absolute neutrophil count of 5200/mm 3 (within normal limits; as per Manroe's chart  ), band cell (immature neutrophil) count to total neutrophil count ratio being zero with no toxic granules, and platelet count of 3,80,000/mm 3 . Baby never had thrombocytopenia, fever or temperature instability during hospital stay. Cardiac troponin T (cTnT) was markedly raised -0.130 ng/ml (N: 0.01 - 0.03 ng/ml) along with creatinine kinase (CPK-MB) - 138 (N: 0-25 U/L) and Lactate dehydrogenase (LDH) - 1089 U/l, on Day 2 of admission. With this background, the diagnosis of myocarditis was apparent. The neonate was managed with free-flow oxygen, decongestive measures, enalapril and immunoglobulin (IVIG) (2 g/kg over 24 h). Baby was never on any inotropic support through the hospital stay. The patient showed marked improvement along with resolution of cardiomegaly and hepatomegaly. Repeat tests after seven days revealed that CPK-MB levels had decreased to 33 U/l, however, cTnT still showed an elevated value of 0.18 ng/ml. By this time, our patient had shown dramatic improvement. The neonate was closely monitored for complications and arrhythmias. After complete clinical resolution patient was discharged on diuretics and enalapril. The parents were counseled for regular follow-up for monitoring of clinical condition, complications, cardiac size and cTnT titers.
|Figure 1: Chest X-ray showing cardiomegaly (CTR-0.8) (left side of figure) on Day 12 (at presentation) and it reduced after treatment (CTR-0.6) (right side of figure), (CTR – Cardiothoracic ratio)|
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| :: Discussion|| |
An isolated VSD rarely presents as CCF before six to eight weeks of life due to high pulmonary vascular resistance preventing volume overload in an early neonatal period.  However, presence of other predisposing factors like prematurity, coexisting structural heart defects, sepsis or anemia may precipitate an early failure. Swiss cheese VSD is a subtype of muscular VSD which rarely closes spontaneously.
Myocarditis is defined as an inflammatory cardiomyopathy causing cardiac dysfunction that occurs in all age groups.  Neonates may present with nonspecific symptoms suggestive of infection including fever, listlessness, poor feeding, or they may present with more ominous signs including apnea, episodic cyanosis, and diaphoresis. Our case had an abrupt, early-onset respiratory distress following a viral prodrome with signs suggestive of CCF, persistent sinus tachycardia and nonspecific ST-T changes on ECG.
Myocarditis is a chronological sequence of three distinct pathophysiological phases. The initial phase is that of an acute insult, followed by immunomodulation and finally the remodeling phase leading to dilated cardiomyopathy (DCM). Generally, after an acute episode one-third of them recover, one-third may deteriorate and the rest may develop DCM, thus warranting regular long-term follow-up. The risk of death and heart transplantation persists for up to 12 years after diagnosis of acute myocarditis in the pediatric population.  An early, aggressive and accurate management can ensure a higher chance of recovery and prevent development of DCM. ,
The optimal strategy for diagnosing myocarditis in children is controversial and based largely on expert opinion.  Elevated cardiac enzymes are the most vital noninvasive markers used for the diagnosis of myocarditis and are more specific than echocardiography. cTnT is a 39 KD subunit of a thin filament of the contractile apparatus of the myocardium which peaks within three to four days of the injury and remains elevated for 6-14 days. Its diagnostic level for pediatric myocarditis is 0.052 ng/ml, with sensitivity and specificity of 71% and 89%, respectively. , Soongswang et al., showed that cTnT level of >0.1 ng/ml (cutoff in adults) would be highly specific for diagnosing myocarditis in children.  cTnT levels are significantly higher in patients with acute myocarditis than in those with moderate to large left-to-right shunt with congestive heart failure.  In our case serum cTnT was markedly elevated above 0.1ng/ml on two different occasions, one week apart, with the second value being much greater than the first one. ,, Thus elevated cTnT, together with increased CPK-MB and LDH, in a case of acute-onset CCF preceded by viral prodrome, in absence of sepsis or renal failure, with ECG changes consistent with myocarditis, conclusively established the diagnosis of postviral myocarditis, in our patient.
The most important aspect in the management of these children includes early and appropriate recognition of the condition and initial aggressive treatment in the form of adequate hemodynamic and ventilator support, if required, followed by close follow-up care. After acute CCF is brought under control, supportive care must be followed by remodeling therapy i.e. angiotensin-converting enzyme (ACE) inhibitors and beta-blockers.  They reverse remodeling of the left ventricle and thus prevent DCM.  Because of the diversity of outcomes in patients with myocarditis and the general lack of dramatic response to treatment, meticulous follow-up of patients to determine their natural history is very important. There are many trials which recommend the use of IVIG in the acute phase, especially in the pediatric population, due to its immunomodulatory effect but no randomized control trial (RCT) is still available to prove its efficacy. ,
Our case report is the first to show such a coexistence of VSD and myocarditis in early neonates. It underscores the fact that CCF in such cases of myocarditis can easily mimic other commoner conditions like VSD and when coexistent can be challenging for the treating physician. Therefore an awareness of clinical presentation of myocarditis, along with its acute timely management and close, long-term follow-up becomes the main factor determining outcome in neonates. We recommend screening for myocarditis in all previously normal babies presenting primarily with cardiogenic symptoms even if a structural heart disease is coexistent early in life.
A simplified algorithm for workup of CCF in a neonate is proposed [Figure 2].
|Figure 2: Algorithm for early-onset CCF in a previously healthy neonate. (CCF – Congestive cardiac failure, PDA – Patent ductus arteriosus, TAPVC – Total anomalous pulmonary venous connection, CPK – Creatinine phosphokinase, ECG - Electrocardiogram)|
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| :: Acknowledgments|| |
We wish to thank Dr. Ravi Rananavare, Dean, T.N. Medical College and BYL Nair Hospital, for permitting us to publish this case
| :: References|| |
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[Figure 1], [Figure 2]