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"ABSTRAKA 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein?s anomaly three years ago, but preferred to be left untreated. The patient was not cyanotic and her vital signs were stable. Her ECG showed incomplete RBBB and prolonged PR-interval. Blood tests revealed mild anemia. Observation of two-dimensional echo with color flow Doppler study showed Ebstein?s anomaly with PFO as additional defects, EF of 57%, LV and LA dilatation, RV atrialization, severe TR, and moderate PH with RVSP of 44.3 mmHg. The patient then underwent elective sectio caesaria at 30 weeks of gestational age; both the mother and her baby were alive and were in good conditions. Problem: there was an increasing breathlessness in this patient so that there was an increasing need to take a decision for her pregnancy.Ebstein?s anomaly is a complicated congenital anomaly. Medical treatment may be followed for many years in patients with mild forms of Ebstein?s anomaly. Surgery should be considered if there is objective evidence of debasement such as significant enlargement of heart size, reduction of systolic function in echocardiography."

"Latar belakang: Anomali Ebstein membutuhkan tindakan pembedahan sebagai tata laksana definitif. Pilihan tindakan pembedahan yang dapat dilakukan adalah perbaikan biventrikular dan nonbiventrikular (1 1⁄2 ventrikel dan univentrikular). Saat ini belum didapatkan algoritma dan faktor prediktor pemilihan tindakan pembedahan yang mencakup seluruh usia.Tujuan: Mengetahui karakteristik pasien yang menjadi faktor prediktor dalam pemilihan tindakan pembedahan pada penderita anomali Ebstein dan keluarannya.Metode: Penelitian ini merupakan studi kohort retrospektif dengan data yang diambil secara total sampling dari pasien anomali Ebstein yang menjalani operasi di Rumah Sakit Pusat Jantung Nasional Harapan Kita sejak Januari 2010–Desember 2023. Variabel bebas yang diteliti adalah usia, aritmia, fungsi ventrikel kanan, regurgitasi trikuspid, cardiothoracic ratio, jarak pergeseran daun katup septal trikuspid, dan skor GOSE, yang distratifikasi berdasarkan tindakan pembedahan yang dilakukan berupa perbaikan biventrikular dan nonbiventrikular terhadap variabel dependen berupa mortalitas.Hasil: Sebanyak 83 subjek dalam penelitian ini, 43 (51,8%) subjek menjalani perbaikan biventrikular dan 40 (48,2%) subjek menjalani perbaikan nonbiventrikular. Tidak terdapat perbedaan bermakna dalam kejadian mortalitas di kedua kelompok perbaikan (p = 0,127). Pada kelompok yang menjalani perbaikan biventrikular, terdapat hubungan yang bermakna secara statistik pada subjek yang mengalami disfungsi ventrikel kanan (p = 0,045, RR 5,1, IK 95%: 1,29-20,45), skor GOSE tinggi (p = 0,042, RR 5,17, IK 95%: 1,08-24,61), dan rerata jarak pergeseran daun katup trikuspid lebih tinggi (p = 0,014) dengan kejadian mortalitas. Pada kelompok yang menjalani perbaikan nonbiventrikular, tidak terdapat perbedaan bermakna secara statistik pada seluruh variabel yang diteliti terhadap kejadian mortalitas. Nilai titik potong jarak pergeseran daun katup trikuspid untuk memprediksi kejadian mortalitas pada perbaikan biventrikular adalah 43,5 mm/m2, dengan sensitivitas 83,3% dan spesifisitas 94,6%.Simpulan: Jarak pergeseran daun katup septal trikuspid berhubungan dengan risiko kejadian mortalitas pada perbaikan biventrikular dan didapatkan nilai titik potong yang baik untuk memprediksi kejadian mortalitas pada perbaikan biventrikular.

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Background: Ebstein anomaly require surgical intervention as definitive treatment. The option for surgical intervention are biventricular repair and non-biventricular repair (one and half ventricle and univentricular). Currently, there is no algorithm and predictors in choosing surgical intervention that could be applicable in all range of age.

Purpose: To identify patient characteristics that can be used as predictors in choosing surgical intervention in Ebstein anomaly and its outcome.

Methods: Retrospective cohort study with total sampling of patients with Ebstein anomaly undergoing surgical intervention at National Cardiovascular Center Harapan Kita from January 2010 until Desember 2023. Independent variables studied were age, arrythmia, right ventricle function, tricuspid regurgitation, cardiothoracic ration, tricuspid septal leaflet displacement, and GOSE score, which were stratified based on the surgical intervention of biventricular or non-biventricular repair, and the dependent variable was mortality.

Result: Out of 83 subjects included in this study, 43 (51.8%) subjects underwent biventricular repair and 40 (48.2%) subject underwent non-biventricular repair. No statistically significant difference were found associated with mortality in both surgical repair group (p = 0.127). In biventricular repair group, subjects with right ventricle dysfunction (p = 0.045, RR 5.1, 95% CI: 1,29-20,45), high GOSE score (p = 0.042, RR 5.17, 95% CI: 1,08-24,61), and higher mean of tricuspid septal leaflet displacement (p = 0,014) has statistically significant association with incidence of mortality. In non- biventricular repair group, all variables have no statistically significant association with incidence of mortality. Tricuspid septal leaflet displacement cut-off point value of 43.5 mm/m2 is best to predict the occurrence of mortality in biventricular repair, with 83.3% sensitivity and 94.6% specificity.

Conclusion: Tricuspid septal leaflet displacement was associated with the occurrence of mortality in biventricular repair and good cut-off point value was obtained to predict mortality in biventricular repair."