초록접수 현황
| 17F-052 | 포럼 발표 |
Impact of Prenatal Diagnosis on the Outcome of Absent Pulmonary Syndrome
You Jung Ok, Bo Sang Kwon, Tae Jin Yun
Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, Ulsan University College of Medicine, Seoul, Republic of Korea
Purpose : We sought to investigate risk factors for mortality in fetuses and infants with absent pulmonary syndrome (APV).
Methods : Fetal and postnatal echocardiographic database and clinical records of all patients with APV who were treated at our center between 2002 and 2016 were reviewed retrospectively.
Results : The cohort consisted of 25 patients; 20 patients (20/25, 80%) with tetralogy of Fallot, two (2/25, 8%) with isolated APV, and three (3/25, 12%) with single ventricle physiology. Nineteen patients were prenatally diagnosed; Ten fetuses were lost to follow-up or terminated in their pregnancy, one fetus died in utero, four fetuses experienced early postsurgical deaths during infancy, and only four patients (4/19, 21%) survived. However, all who were diagnosed postnatally survived. Two fetuses with dysplastic tricuspid valve had hydrops fetalis. No differences were found between the survivors and non-survivors in indexed main, right, and left pulmonary artery diameters, right ventricle size/ left ventricular size, right atrial diameter / left atrial diameter, tricuspid annular size/ mitral annular size and peak velocity of pulmonary stenosis or regurgitation on fetal echocardiography. Pulmonary valve annulus size to aortic valve annulus size ratio was larger in non-survivors without statistical significance (1.01 ± 0.35 vs. 0.78 ± 0.11; P = 0.183) on fetal echocardiogram. Underlying single ventricular physiology (R2 =0.355, P =0.019) and right or left ventricular dysfunction (R2=0.5, P =0.003) predicted postsurgical death.
Conclusion : The outcomes of APV appear poor, especially in patients with prenatal diagnosis, single ventricular physiology, and right or left ventricular dysfunction.
Methods : Fetal and postnatal echocardiographic database and clinical records of all patients with APV who were treated at our center between 2002 and 2016 were reviewed retrospectively.
Results : The cohort consisted of 25 patients; 20 patients (20/25, 80%) with tetralogy of Fallot, two (2/25, 8%) with isolated APV, and three (3/25, 12%) with single ventricle physiology. Nineteen patients were prenatally diagnosed; Ten fetuses were lost to follow-up or terminated in their pregnancy, one fetus died in utero, four fetuses experienced early postsurgical deaths during infancy, and only four patients (4/19, 21%) survived. However, all who were diagnosed postnatally survived. Two fetuses with dysplastic tricuspid valve had hydrops fetalis. No differences were found between the survivors and non-survivors in indexed main, right, and left pulmonary artery diameters, right ventricle size/ left ventricular size, right atrial diameter / left atrial diameter, tricuspid annular size/ mitral annular size and peak velocity of pulmonary stenosis or regurgitation on fetal echocardiography. Pulmonary valve annulus size to aortic valve annulus size ratio was larger in non-survivors without statistical significance (1.01 ± 0.35 vs. 0.78 ± 0.11; P = 0.183) on fetal echocardiogram. Underlying single ventricular physiology (R2 =0.355, P =0.019) and right or left ventricular dysfunction (R2=0.5, P =0.003) predicted postsurgical death.
Conclusion : The outcomes of APV appear poor, especially in patients with prenatal diagnosis, single ventricular physiology, and right or left ventricular dysfunction.
책임저자: Tae-Jin Yun
Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, Ulsan University College of Medicine, Seoul, Republic of Korea
발표자: You Jung Ok, E-mail : keinmiel@hanmail.net