1.A Rare Case of Lethal Prenatal-Onset Infantile Cortical Hyperostosis
Susan Taejung KIM ; Hyeseon KIM ; Hyun Ho KIM ; Na Hyun LEE ; Yeaseul HAN ; Se In SUNG ; Yun Sil CHANG ; Won Soon PARK
Yonsei Medical Journal 2019;60(5):484-486
Infantile cortical hyperostosis, or Caffey's disease, usually presents with typical radiological features of soft tissue swelling and cortical thickening of the underlying bone. The disease can be fatal when it presents antenatally, especially before a gestational age of 35 weeks. This fatal, premature form of the disease is known to occur in various ethnic groups around the globe, and approximately 30 cases have been reported in English literature. This paper is unique in that it is the first paper to report a lethal form of prenatal-type infantile cortical hyperostosis diagnosed in South Korea. Born at gestational age of 27 weeks and 4 days, the patient had typical features of polyhydramnios, anasarca, hyperostosis of multiple bones, micrognathia, pulmonary hypoplasia, and hepatomegaly. The patient was hypotonic, and due to pulmonary hypoplasia and persistent pulmonary hypertension, had to be supported with high frequency ventilation throughout the entire hospital course. Due to the disease entity itself, as well as prolonged parenteral nutrition, liver failure progressed, and the patient expired on day 38 when uncontrolled septic shock was superimposed. The chromosome karyotype of the patient was normal, 46, XX, and COL1A1 gene mutation was not detected.
Edema
;
Ethnic Groups
;
Gestational Age
;
Hepatomegaly
;
High-Frequency Ventilation
;
Humans
;
Hyperostosis
;
Hyperostosis, Cortical, Congenital
;
Hypertension, Pulmonary
;
Infant, Newborn
;
Infant, Premature
;
Karyotype
;
Korea
;
Liver Failure
;
Micrognathism
;
Parenteral Nutrition
;
Polyhydramnios
;
Shock, Septic
2.The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease
Susan Taejung KIM ; Jinyoung SONG ; June HUH ; I Seok KANG ; Ji Hyuk YANG ; Tae Gook JUN ; Soo young OH ; Suk Joo CHOI ; Cheong Rae ROH
Journal of Korean Medical Science 2019;34(24):e170-
BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.
Aorta, Thoracic
;
Aortic Coarctation
;
Arrhythmias, Cardiac
;
Arteries
;
Birth Rate
;
Echocardiography
;
Fetus
;
Heart Defects, Congenital
;
Heart Diseases
;
Hypoplastic Left Heart Syndrome
;
Parturition
;
Prenatal Diagnosis
;
Pulmonary Atresia
;
Surgeons
;
Tetralogy of Fallot
3.Late Outcomes of Pediatric and Congenital Heart Disease Patients Following Cardiac Resynchronization Therapy
Jeong Eun AHN ; Susan Taejung KIM ; Hye Won KWON ; Sang Yun LEE ; Gi Beom KIM ; Jae Gun KWAK ; Woong Han KIM ; Mi Kyoung SONG ; Eun Jung BAE
Korean Circulation Journal 2022;52(12):865-875
Background and Objectives:
Cardiac resynchronization therapy (CRT) is an effective treatment for heart failure. However, in pediatric and congenital heart disease (CHD) patients, current adult indications cannot be directly applied because of heterogeneity in anatomy and diagnosis. Therefore, CRT responses and clinical outcomes in these patients were investigated to derive possible candidates for CRT.
Methods:
This study retrospectively analyzed 16 pediatric and CHD patients who underwent CRT implantation at a single center in early (0.7±0.2 year) and late (4.7±0.3 years) follow-up period after CRT.
Results:
The median age at CRT implantation was 2.5 (0.3–37.2) years, and median follow-up duration was 6.3 (0.1–13.6) years. Thirteen had non-transvenous CRT. Two had congenital complete atrioventricular (AV) block with previous right ventricular pacing, 5 had dilated cardiomyopathy (DCM) with left bundle branch block, and 9 had CHD. The mean ejection fraction of the systemic ventricle increased from 28.1±10.0% to 44.3±21.0% (p=0.003) in early and 51.8±16.3% (p=0.012) in late outcome. The mean functional class improved from 3.1±0.9 to 1.8±1.1 after CRT (p=0.003). Twelve patients (75%) showed improvement in ventricular function or functional class after CRT. Proportion of responders differed between patients without CHD (2/2 patients with complete AV block and 5/5 with DCM, 100%) and those with CHD (5/9, 56%), although statistical significance was not reached (p=0.088).
Conclusions
CRT improved ventricular function and functional status according to the underlying condition in pediatric and CHD patients. However, further large and longer-term studies are needed to establish the guideline for the patient selection of CRT in these patients.