Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare, combined Mullerian and Mesonephric duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. We present the case of an otherwise healthy 16-year-old female with acute urinary retention secondary to HWWS. The diagnosis was established with abdominal ultrasound and Magnetic Resonance Imaging (MRI). The patient subsequently underwent surgical resection of the vaginal septum resulting in relief of obstruction. Clinical symptoms in patients with HWWS typically present after menarche with progressive hematometra causing pain and compression of localized structures. Even though ultrasound can help in the diagnosis, MRI is the best choice of imaging for the visualization of these anomalies. The diagnosis of HWWS is important to consider in young females of reproductive age presenting with symptoms of obstruction of adjacent structures. Our patient presented with acute urinary retention which is a rare symptom in this entity. A high index of clinical suspicion and awareness of the syndrome are required to make a speedy diagnosis and prevent future complications.

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Introduction: Ventriculo-arterial coupling (VAC) ratio (normal value: 0.6 - 1.2) is worse in children with dilated cardiomyopathy compared to controls and is associated with poor heart failure (HF) outcomes. It has not been studied in patients on oral HF medications. Our aim was to study VAC ratio as a measure of response to therapy and its ability to predict worse outcomes in this population. Methods: A retrospective study of DCM patients < 21 years on oral heart failure medications was conducted between 2010 and 2022 at the Children's Hospital At Montefiore. Medication data, NYHA functional class, and outcomes (mechanical circulatory support, transplantation, or death) were collected. A single observer analyzed the initial and the last echocardiograms on optimal HF therapy to calculate arterial elastance (EA), ventricular elastance (EES), and VAC ratio. Patients with and without outcomes were compared using chi-square & Mann Whitney tests, as appropriate. Results: Thirty-one patients (median age at diagnosis 1.25 (IQR 7.7) years) were followed for a median of 4.92 (IQR 11) years. There was a significant improvement in the VAC ratio (mean 1.85 vs 1.46, SEM 0.17, P=0.02) from diagnosis to the last follow-up with a median change of -13.5%. There was a statistically significant association between the percent change in VAC and change in the NYHA class (P=0.0001). Outcome events occurred in 7 patients; one required mechanical circulatory support, two died, and four underwent transplants. Patients with an outcome event had a worse VAC ratio both at diagnosis (P=0.03) and at the last follow-up (P=0.0001) and a worse NYHA class (P=0.001) at the last follow-up (Table). Conclusion: VAC is a potential biomarker for response to therapy and is associated with poor outcomes in pediatric DCM. It provides mechanistic insights into HF in this population.