Genetic errors and teratogenic events during embryonic development can lead to congenital abnormalities of the female reproductive tract. Many patients are asymptomatic, while some have major abnormalities that can cause severe impairment of menstrual and reproductive functions. This case report focuses on Müllerian anomaly Class III, uterine didelphys, along with obstructed hemivagina and ipsilateral renal agenesis (the Herlyn–Werner–Wunderlich syndrome [HWWs]). The patient presented with cyclic pelvic pain due to the hematometra and hematocolpos. She initially underwent resection of the vaginal septum but had a recurrence of obstructive symptoms more than a year after vaginal surgery was performed. This prompted further evaluation with magnetic resonance imaging revealing cystic dilatation of the right uterine horn and a hypoplastic right vagina appearing to end blindly. The patient subsequently underwent hemihysterectomy. The right hemiuterus was noted to have a depression on its thickened inferior aspect, but no cervix was identified. This case is a variant of the classic HWWS, as there was cervical atresia on the right uterine corpus. A thorough preoperative evaluation and accurate intraoperative assessment of patients with Müllerian anomalies can decrease misdiagnoses, guide appropriate intervention, and decrease the risk of future reproductive complications.
Cervical atresia ; uterine didelphys

Between 1975 and 1981, 171 patients(pts) had ventricular septal defect, as isolated one or one part of various multiple congenital malformations, at catheterization. Ninety of these pts(52.5%) had isolated VSD, and 45(26%) had tetralogy of Fallot. Other associated anomalies include ASD, PDA, AI, MI, TGA, DOLV, COA, PS, sinus valsalva rupture, tricuspid atresia, pulm. atresia, single atrium and bilat. SVC. Fifty-two VSD patients were operated upon with conventional extracorporeal circulation. In these operated cases, Kirklin's type II VSD were most common(24/52, 46%); followed by type I(12/52), type III(11/52), type IV(1/52), multiple VSD(2/52) and LV-RA shunt(1/52). The hospital mortality was 7.7%(4/52). Transient complete heart block occurred in 3 pts. Of the survivors, 44% had RBBB(21/48) and 7 of them had associated left anterior hemiblock(LAH). In all 7 pts with immediate post-operative LAH, left axis deviation become normalized within 5 months after operation(post-op. 1 day-5Mo.). We also analized pre-operative ECG, chest PA and hemodynamic data of VSD, which showed us the close correlation between ventricular hypertrophy patterns in ECG and hemo dynamic datas(LVH with Qp/Qs, and RVH with peak systolic pressure of pulmonary artery or Pp/Ps), and then correlation with post-operative results.
Axis, Cervical Vertebra ; Blood Pressure ; Catheterization ; Catheters ; Electrocardiography ; Extracorporeal Circulation ; Heart Block ; Heart Septal Defects, Ventricular* ; Hemodynamics ; Hospital Mortality ; Humans ; Hypertrophy ; Pulmonary Artery ; Rupture ; Survivors ; Tetralogy of Fallot ; Thorax ; Tricuspid Atresia

PURPOSE: Anomalous connection of coronary artery can result in damage of myocardial perfusion, myocardial infarction, or congestive heart failure. Echocardiography has been used to demonstrate coronary artery anomalies such as coronary artery fistula, anomalous origin of the left coronary artery from the pulmonary artery, and aneurysm of the coronary arteries in Kawasaki disease. Definitive diagnosis relies on cardiac catheterization The objectives of this study were to know the diagnostic sensitivity of echocardiography in detecting abnormal coronary artery connections, and identify the direction of blood flow by pulsed Doppler echocardiography in these patients. METHODS: Evidence of abnormal connections were found during echocardiographic examination in 25 patients(anomalous origin of left coronary artery from the pulmonary artery, coronary artery fistula, right ventricular to coronary artery sinusoids in pulmonary atresia with intact ventricular septum, and left ventricular to coronary artery sinusoids in aortic atresia with intact venticular septum). Two-dimensional imaging, color and pulsed Doppler echocardiography were performed in supine or left decubitus position from parasternal, apical, and subcostal views. The internal dimension of the coronary arteries and aortic root were measured at the level of aortic annulus during systole from the parastenal short axis view. RESULTS: 1) Diagnostic sensitivity by echocardiography in anomalous origin of the left coronary artery from the pulmonary artery was 85.7%(6/7). The direction of flow was from the left coronary artery into pulmonary artery during systole and reversed flow in diastole in 4 patients. There was continuous flow from the left coronary artery to the pulmonary artery in 1 patient. 2) Diagnostic sensitivity by echocardiography in congenital coronary artery fistula was 85.7% (6/7), and in acquired coronary artery fistula was 100%(4/4). There was continous systolic and diastolic flow from the coronary artery into the right ventricle or pulmonary artery by pulsed Doppler in all patients. 3) Diagnostic sensitivity in coronary sinusoid was 100% (7/7). Pulsed Doppler echocardiography revealed systolic flow from the right or left ventricle into coronary circulation during systole, and reversed flow during diastole. CONCLUSIONS: Color and pulsed Doppler echocardiography is extremely useful in detecting abnormal coronary artery connections. Although the sensitivity of echocardiography to coronary artery abnormalities is not 100%, there was no false positive. Cardiac catheterization may be necessary to confirm the diagnosis, but echocardiography is a helpful diagnostic tool in high risk infnats.
Aneurysm ; Axis, Cervical Vertebra ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Circulation ; Coronary Vessels* ; Diagnosis ; Diastole ; Echocardiography ; Echocardiography, Doppler, Color* ; Echocardiography, Doppler, Pulsed ; Fistula ; Heart Failure ; Heart Ventricles ; Humans ; Mucocutaneous Lymph Node Syndrome ; Myocardial Infarction ; Perfusion ; Pulmonary Artery ; Pulmonary Atresia ; Systole ; Ventricular Septum