ECTOPIA CORDIS

oleh : dr. Ali Nafiah Nasution

Introduction

Ectopia cordis is an extremely rare congenital abnormality, occurring in 5.5 to 7.9 per 1 million live births.^1-8 The defect is characterized by partial or complete displacement of the heart out of the thoracic cavity. This anomaly is classified into five types: cervical, cervicothoracic, thoracic, abdominal, and thoracoabdominal.^1,5,9 The two most common forms of ectopia cordis are the thoracic and thoracoabdominal type.^1,4,9 The latter is frequently associated with Cantrell’s pentalogy, first described by Cantrell et al. in 1958, which include defect of the lower sternum, deficiency of the diaphragm, defect of diaphragmatic pericardium, defect of the anterior abdominal wall, and intracardiac defects.^1,3,5,7-17

Previous reports suggest a poor prognosis for patients with ectopia cordis, particularly in the presence of thoracic ectopia cordis and significant heart defects.^{1,2,7,9,15,18,19} More recently, with the advances in the medical field and surgical techniques, more patients born with this medical condition have been successfully treated and have survived. In general, the goal of the initial management is directed at providing coverage of the bare heart with skin or synthetic material without causing hemodynamic embarrassment. Later, subsequent operations to repair the intracardiac defects and to reconstruct the chest wall can be done.^1,5,9,19 In this report, I present a case of ectopia cordis and an overview of its management in the medical literature.

Case Report

A 3500-g male infant was born via a lower segment caesarian section to a young 26 years primigravida mother at 40 weeks of gestation following an uncomplicated pregnancy. There was no family history of congenital defects. The baby was breathing spontaneously. Physical examination showed a purple mass just above the umbilical insertion pulsating with the heart beat. Further physical examination was unremarkable.

Electrocardiography showed sinus rhythm with normal axis and right ventricular hypertrophy. Chest X-ray showed dextrocardia. Echocardiography showed a dextroposition of the heart. There was ASD and VSD with a left-to right shunt and pulmonary valve stenosis. CT thorax showed dextrocardia and ectopia cordis with the abdominalis hernia.

Patient in stable condition and planned to have repaired surgery for the defects.

Reaktivasi pada Penyakit Jantung Rematik

oleh: Tengku Winda Ardini


Pendahuluan

Demam rematik (DR) dan penyakit jantung rematik (PJR) masih merupakan masalah kesehatan yang penting di negara berkembang, termasuk Indonesia. Demam rematik merupakan penyebab utama penyakit jantung didapat pada usia anak 5 tahun sampai usia dewasa muda di negara berkembang dengan keadaan sosial ekonomi rendah, dan jarang ditemukan pada anak di bawah usia 5 tahun. Pada tahun 1944 diperkirakan diseluruh dunia terdapat 12 juta penderita DR dan PJR dan sekitar 3 juta mengalami gagal jantung dan memerlukan rawat inap berulang di rumah sakit. Angka kejadian DR/PJR di Indonesia dan negara-negara berkembang lainnya masih tinggi. Pada negara berkembang diperkirakan angka DR dan PJR mencapai 20 juta orang dan merupakan penyebab utama kematian oleh karena penyakit kardiovaskular dalam 50 tahun pertama kehidupan, hal ini memperlihatkan mortalitas karena DR dan PJR masih merupakan problem dan kematian karena DR akut terdapat pada anak dan dewasa muda. Prevalens PJR di Indonesia diperkirakan sebesar 0,3-0,8 per 1000 anak berusia 5-15 tahun berdasarkan data terakhir tahun 1981-1990.1-4