Thirty-Day Readmission Rate and Healthcare Economic Effects of Patients With Transcatheter Aortic Valve Replacement and Coexisting Chronic Congestive Heart Failure.

Transcatheter aortic valve replacement (TAVR) procedures have increased since adoption in 2010. Readmission for TAVR patients with underlying chronic congestive heart failure (cCHF) remains challenging. Therefore, we sought to determine the 30-day readmission rate (30-DRr) of patients who undergo TAVR & co-existing cCHF and its impact on mortality & healthcare utilization in the United States. We performed a retrospective study using the national readmission database year 2017 and 2018. The patients studied were discharged with TAVR as a principal diagnosis and underlying cCHF as a secondary diagnosis according to ICD-10 codes. The primary outcome was a 30-day readmission rate and mortality, while secondary outcomes were the most common diagnoses for readmission, and resource utilization. A total of 76,892 index hospitalization for TAVR with coexisting cCHF: mean age was 79.7 years [SD: ± 2], and 54.5% of patients were males. In-hospital mortality rate for index admission was 1.63%. The 30-DRr was 9.5%. Among the group of readmitted patients, in-hospital mortality rate was 3.13%. Readmission mortality showed a statistically significant increase compared to index mortality (3.13% vs 1.63%, adjusted P ≤ 0.001, aOR: 2.1, 95% CI: 1.6-2.9). The total healthcare in-hospital economic spending was $94.4 million, and total patient charge of $412 million. Approximately 1 in 10 patients who underwent TAVR with underlying cCHF had 30-DRr, with subsequent readmissions associated with increased healthcare spending. Readmission mortality showed a statistically significant increase when compared to index mortality. TAVR patients with cCHF are a vulnerable subset requiring additional outpatient care.

Diagnosis of GATA2 haplo-insufficiency in a young woman prompted by pancytopenia with deficiencies of B-cell and dendritic cell development.

BACKGROUND: GATA2 deficiency presents with a spectrum of phenotypes including increased susceptibility to viral and bacterial infections, multi-lineage cytopenias, aplastic anemia, leukemic transformation and lymphedema. Allogeneic transplantation is only curative therapy for GATA2 deficiency, but is associated with significant treatment related morbidity and mortality. Given the spectrum of clinical presentation, accurate diagnosis of GATA2 deficiency is necessary to identify patients early in their disease course when allogeneic bone marrow transplantation may be of clinical benefit. CASE PRESENTATION: In this report, we present a GATA2 mutation diagnosed in 23-year-old woman presenting with pancytopenia, recurring oral blisters, fatigue and chronic pain. We describe markedly low levels of mature B-cells in the blood and bone marrow and the absence of detectable blood dendritic cells with normal serum immunoglobulin levels and normal numbers of marrow plasma cells. She was ultimately diagnosed with GATA2 haplo-insufficiency due to a GATA2 germ-line mutation and underwent a successful allogeneic bone marrow transplant from a 10/10 HLA matched unrelated donor. CONCLUSIONS: The case illustrates the diagnostic difficulties in identifying GATA2 deficiencies and the importance of family history and genetic testing. GATA2 plays an important role in B-cell and dendritic cell development, and decreased numbers of those cells is a characteristic feature that should prompt consideration of GATA2 deficiency in a patient with pancytopenia. Maturation of B-cells to long-lived plasma cells is relatively unaffected in GATA2 deficiency. Allogeneic stem cell transplantation can correct immune-deficiencies and prevent leukemic transformation in patients with GATA2 deficiency.