|Year : 2014 | Volume
| Issue : 3 | Page : 161-163
Transient severe isolated right ventricular hypertrophy in neonates
Hayrullah Alp1, Zehra Karataş2, Tamer Baysal2
1 Pediatric Cardiology Clinic, Malatya State Hospital, Malatya, Turkey
2 Department of Pediatric Cardiology, Meram School of Medicine Hospital, Necmettin Erbakan University, Meram, Konya, Turkey
|Date of Web Publication||8-Sep-2014|
Dr. Hayrullah Alp
Pediatric Cardiology Clinic, Malatya State Hospital, Malatya
Source of Support: None, Conflict of Interest: None
Isolated right ventricular hypertrophy (RVH) is a rare form of hypertrophic cardiomyopathy and premature closure of the patent ductus arteriosus in utero is a probable etiologic factor. We reported transient isolated RVH in three neonates and ventricular hypertrophy resolved within 8-10 weeks period without any specific therapy in all cases.
Keywords: Newborn, premature closure of ductus arteriosus, transient, ventricular hypertrophy
|How to cite this article:|
Alp H, Karataş Z, Baysal T. Transient severe isolated right ventricular hypertrophy in neonates. J Clin Neonatol 2014;3:161-3
| Introduction|| |
Transient right ventricular hypertrophy (RVH) is a rare anomaly which is seen during neonatal period.  Such metabolic diseases, fetal distress, maternal diabetes, non-steroidal anti-inflammatory drug (NSAID) use during pregnancy and dexamethasone treatment in premature newborns for bronchopulmonary dysplasia may cause transient and/or isolated RVH in newborn period.  Furthermore, premature closure of the ductus arteriosus is a rare cause of isolated RVH and in utero it has been shown to be resulted in pulmonary arterial hypertension. ,,,, In utero closure of the fetal ductus arteriosus may occur spontaneously with an unknown etiology, but it is mostly caused by pharmacological agents. , We have reported three newborns presenting with isolated RVH and also we reviewed the current English pediatric cardiologic literature.
| Case Report|| |
Two male and a female neonates, appropriate for gestational age, on born at 38-40 weeks gestation by caesarian section as the first, third and second pregnancies, were evaluated in our clinic for cyanosis. There was no history of diabetes mellitus, hypertrophic cardiomyopathy, NSAID or steroid drug usage and consumption of polyphenol-rich substances in the mothers of either baby. Furthermore, any perinatal stress was not detected. All babies had higher 1, 5 and 10 min Apgar scores after birth. However, all babies developed respiratory distress and cyanosis in which arterial blood gases revealed systemic hypoxia with oxygen saturation of 70, 76 and 80%, respectively. Electrocardiograms showed sinus tachycardia and significant RVH. Echocardiographic investigation which was performed on the 1 st day of life, revealed marked hypertrophy of RV free wall (8, 9 and 11 mm in diastole, respectively), interventricular septum (7, 8 and 8.5 mm in diastole, respectively) and a small RV cavity (1.85, 1.63 and 1.69 cm 2 in diastole, respectively) [Figure 1]a. The posterior wall of the left ventricle (LV) and the cavity were in normal dimensions. Systolic blood pressures' of the newborns were 60, 65 and 65 mmHg, respectively and color Doppler echocardiography revealed severe tricuspid regurgitation with pressure gradients of 45, 50 and 50 mmHg, respectively which refer to RV pressure. However, no shunt was detected across the ductus arteriosus in both babies which was diagnosed as closed and the LV functions were normal. No structural cardiac abnormality was detected instead of patent oval foramen with left to right shunting in all babies. Neither baby had received steroids instead of supportive treatment with intravenous fluid and maintenance of temperature. Their condition improved gradually and during the follow-up at 8-10 weeks, the babies were stable with oxygen saturation of 95-97%. Also, echocardiography revealed complete regression of RVH and normal pulmonary arterial pressures [Figure 1]b. All babies have been following up in our clinic for 5, 6 and 9 months without any complication.
|Figure 1: Apical four-chamber view on the 1st day of life that revealed (a) severe isolated RVH with reduced RV cavity and normal LV. (b) Apical four-chamber view at 10 weeks of life, which showed complete regression of RVH. RVH: Right ventricular hypertrophy, RV: Right ventricle, LV: Left ventricle|
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| Discussion|| |
Medline review of recent English pediatric cardiologic literature revealed that few cases of transient isolated RVH have been reported ,,,,,,, and comparison of these cases was given in [Table 1]. Some congenital heart anomalies, such as pulmonary stenosis, tetralogy of Fallot or truncus arteriosus, may cause RVH.  Transient RVH may also be secondary to metabolic diseases, maternal diabetes, dexamethasone therapy in premature newborns for bronchopulmonary dysplasia  , NSAID  such as diclofenac  and cyclo-oxygenase inhibitors such as nimesulide  usage during pregnancy. In addition, premature closure of ductus arteriosus is a rare cause of transient isolated RVH , which is the presumed etiology of our patients, based on the echocardiographic finding which was performed on the 1 st day of life. The exact reason for idiopathic or spontaneous intrauterine closure of the ductus arteriosus is not known exactly. , However, it has been showed that the following maternal administrations of the prostaglandin synthetase inhibitors such as aspirin, glucocorticoids and NSAIDs during pregnancy may cause intrauterine closure of the human ductus arteriosus. ,,,, Furthermore, there was no history of these administrations in our mothers.
|Table 1: Comparison of reported cases with transient isolated RVH due to premature closure of ductus arteriosus in recent English literature|
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Prostaglandins E1 and E2 play a major role in maintaining the persistence of ductus arteriosus during fetal period. Hence, prostaglandins synthesize inhibitors such as diclofenac  and nimesulide  cause the constriction of ductus arteriosus. Furthermore, maternal consumption of polyphenol-rich substances may constrict the fetal ductus arteriosus.  Most of the reports have showed that transient isolated RVH resolves spontaneously without any specific medications up to 8 weeks. ,,,, Similarly, in our both babies RVH resolved spontaneously in 8-10 weeks. We suggest that the time of resolution may also be associated with the severity of hypertrophy and pulmonary hypertension.
In contrast, another postulated mechanism about RVH is an increase in RV systolic pressure, or pulmonary hypertension as the primary stimulus causing RV pressure load and RVH. All the supportive medications such as intermittent positive-pressure ventilation or nitrite oxide inhalation, aims to decrease pulmonary pressure in these patients. However, most of the patients resolve spontaneously without these supportive medications.
In summary, premature closure of ductus arteriosus can cause transient isolated RVH. Close monitoring of babies with isolated RVH may help us to prevent cardiac failure. Transient isolated RVH has a good prognosis and resolves spontaneously in 8-10 weeks instead of its severity.
| References|| |
|1.||Werner JC, Sicard RE, Hansen TW, Solomon E, Cowett RM, Oh W. Hypertrophic cardiomyopathy associated with dexamethasone therapy for bronchopulmonary dysplasia. J Pediatr 1992;120:286-91. |
|2.||Gewillig M, Brown SC, De Catte L, Debeer A, Eyskens B, Cossey V, et al. Premature foetal closure of the arterial duct: Clinical presentations and outcome. Eur Heart J 2009;30:1530-6. |
|3.||Luchese S, Mânica JL, Zielinsky P. Intrauterine ductus arteriosus constriction: Analysis of a historic cohort of 20 cases. Arq Bras Cardiol 2003;81:405-10, 399-404. |
|4.||Trevett TN Jr, Cotton J. Idiopathic constriction of the fetal ductus arteriosus. Ultrasound Obstet Gynecol 2004;23:517-9. |
|5.||Long WE, Wilson AD, Srinivasan S, Seeger KJ, Maginot KR. In utero premature closure of the ductus arteriosus presenting as isolated right ventricular hypertrophy. WMJ 2009;108:370-2. |
|6.||Tomar M, Radhakrishnan S, Shrivastava S. Transient severe isolated RVH in neonates. Cardiol Young 2003;13:e384-6. |
|7.||Auer M, Brezinka C, Eller P, Luze K, Schweigmann U, Schwärzler P. Prenatal diagnosis of intrauterine premature closure of the ductus arteriosus following maternal diclofenac application. Ultrasound Obstet Gynecol 2004;23:513-6. |
|8.||Paladini D, Marasini M, Volpe P. Severe ductal constriction in the third-trimester fetus following maternal self-medication with nimesulide. Ultrasound Obstet Gynecol 2005;25:357-61. |
|9.||Del Pasqua A, Iadanza A, Pierli C. Unexplained severe transient hypertrophy of the right ventricle in a newborn. J Cardiovasc Med (Hagerstown) 2007;8:311-2. |
|10.||Zielinsky P, Piccoli AL Jr, Manica JL, Nicoloso LH. New insights on fetal ductal constriction: Role of maternal ingestion of polyphenol-rich foods. Expert Rev Cardiovasc Ther 2010;8:291-8. |