Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome.

An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.

Utilization Practices of Inferior Vena Cava Filters at an Academic Medical Center.

INTRODUCTION:  Anticoagulation is the mainstay of management for patients with venous thromboembolism (VTE), which includes deep vein thrombosis (DVT) and pulmonary embolism (PE). Inferior vena cava (IVC) filters are indicated in select patients who are not candidates for anticoagulation. There is a lack of quality evidence supporting other indications. In addition, long-term benefits and safety profiles of IVC filters have not been established. We investigated the utilization practice of IVC filters in a contemporary series of patients in a tertiary academic medical center. METHODOLOGY:  A retrospective review of 200 patients who received IVC filters at Virginia Commonwealth University (VCU) Medical Center in the years 2017 and 2018 was conducted. Adult patients 18 years of age or older with or without cancer were included, and patients were selected consecutively until data on 200 patients were collected. Data on patient demographics, an indication of IVC filter placement, filter retrieval rate, and re-thrombosis events over a median follow-up period of nine months were extracted from the electronic medical record and analyzed. RESULTS: A total of 200 patients (105 male and 95 female) were included with a median age of 61 years (range 17-92 years). Of the 200 patients, 97 (48.5%) had a DVT, 28 (14%) had a PE, 73 (36.5%) had both a PE and DVT, and 2 (1%) had thrombosis at other sites. A total of 130 (65%) patients had an IVC filter placed because of a contraindication to anticoagulation, while 70 (35%) had an IVC filter placed for other nonstandard indications, which included new or worsening VTE despite anticoagulation, recent VTE who must have anticoagulation held during surgery, primary prevention in high-risk patients, and extensive disease burden among other reasons. Seventy-two (36%) patients had active malignancy at the time of filter placement, and 64 (32%) were lost to follow-up. Of the 119 patients who were potentially eligible for filter retrieval, 55 (46%) patients had their IVC filters removed at a median of five months after insertion. Of the 55 patients who had IVC filters removed, 8 (14.5%) patients experienced a re-thrombosis event within a median follow-up of 39 months. Of the 145 patients who still had their filter in place at the time of death or last follow-up, 5 (3.4%) patients experienced a re-thrombosis event within a median follow-up of three months. CONCLUSIONS:  One-third of the patients in this series had an IVC filter placed without a standard indication, and less than half of them had the IVC filters removed within one year of placement. Additionally, one-third of the patients were lost to follow-up, highlighting the need for improved structured follow-up programs and education among both patients and providers regarding the indications for placement and retrieval to minimize complications.

Improved Survival of Young Patients With Breast Cancer 40 Years and Younger at Diagnosis.

PURPOSE: Around 50% of patients with breast cancer in low- or middle-income countries are younger than 50 years, a poor prognostic variable. We report the outcome of patients with breast cancer 40 years and younger. METHODS: We reviewed 386 patients with breast cancer 40 years and younger and retrieved demographic, clinicopathologic, treatment-related, disease progression, and survival data from electronic medical records. RESULTS: The median age at diagnosis was 36 years, and infiltrating ductal carcinoma was present in 94.3% of patients, infiltrating lobular carcinoma in 1.3%, and ductal carcinoma in situ in 4.4%. Grade 1 disease was present in 8.5% of patients, grade 2 in 35.5%, and grade 3 in 53.4%; 25.1% had human epidermal growth factor receptor 2 (HER2)-positive, 74.6% had hormone receptor (HR)+, and 16.6% had triple-negative breast cancer. Early breast cancer (EBC) constituted 63.6% (stage I, 22.4%; stage II, 41.2%) of patients, whereas 23.2% had stage III, and 13.2% had metastatic disease at diagnosis. Of patients with EBC, 51% had partial mastectomy and 49.0% had total mastectomy. And 77.1% had chemotherapy with or without anti-HER2 therapy. All HR+ patients received adjuvant hormonal therapy. The disease-free survival at 5 years was 72.5% and 55.9% at 10 years. The overall survival (OS) was 89.4% at 5 years and 76% at 10 years. Patients with stages I/II had an OS of 96.0% at 5 years and 87.1% at 10 years. Patients with stage III had an OS of 88.3% at 5 years and 68.7% at 10 years. The OS of patients with stage IV was 64.5% at 5 years and 48.4% at 10 years. CONCLUSION: We report survival rates of 89% at 5 years and 76% at 10 years with modern multidisciplinary management. Best results were seen in EBC: OS rates of 96% and 87% at 5 years and 10 years.

Sotalol as an effective adjunct therapy in the management of supraventricular tachycardia induced fetal hydrops fetalis.

Sustained fetal supraventricular tachycardia (SVT) complicated by hydrops fetalis carries a significant risk of morbidity and mortality. While there is no clear consensus on first- and second-line therapy options for the management of fetal SVT with or without hydrops fetalis, there exists significant nonrandomized experience with a number of antiarrhythmic agents that has founded the basis for management. Furthermore, recently published meta-analyses and ongoing multicenter prospective studies have aimed to bridge the gap in the literature. We report two cases of sustained fetal SVT with severe secondary hydrops fetalis managed successfully with flecainide-sotalol combination therapy in one case and sotalol-digoxin combination therapy in the second and review the literature for the management of fetal SVT.