Anderson JB, Beekman RH 3rd, Kugler JD, Rosenthal GL, Jenkins KJ, Klitzner TS, Martin GR, Neish SR, Brown DW, Mangeot C, King E, Peterson LE, Provost L, Lannon C, National Pediatric Cardiology Quality Improvement C. Improvement in Interstage Survival in a National Pediatric Cardiology Learning Network. Circ Cardiovasc Qual Outcomes. 2015 Jul;8(4):428-36.
This project demonstrated for the first time the power of a collaborative learning network to improve outcomes in infants with a rare disease, hypoplastic left heart syndrome (HLHS). Using improvement methods, and the participation of 60 surgical centers, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) demonstrated improved discharge and outpatient clinic processes that resulted in a 40% reduction in interstage mortality in patients with HLHS in this collaborative. This work was the result of collaboration among cardiologists, nurses, and parents to co-produce improvement efforts that ultimately resulted in these improved outcomes.
Niss O, Quinn CT, Lane A, Daily J, Khoury PR, Bakeer N, Kimball TR, Towbin JA, Malik P, Taylor MD. Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease. JACC Cardiovasc Imaging. 2016 Mar;9(3):243-52.
Research associates sickle cell disease with significant cardiopulmonary morbidity, including an increased rate of unexplained sudden death. This paper describes a novel paradigm for the cardiac involvement in sickle cell disease as a form of cardiomyopathy with a restrictive phenotype. The paper has two parts: an extensive meta-analysis of previously published cardiac imaging studies, and a detailed echocardiographic study from our institution that together show the unique high output restrictive physiology in this patient group. These findings are the preliminary data used to design the current clinical trial of cardiac MR and serologic biomarkers to further delineate the cardiopulmonary phenotype of sickle cell disease.
Towbin JA, Lorts A, Jefferies JL. Left ventricular non-compaction cardiomyopathy. Lancet. 2015 Aug 22;386(9995):813-25.
This report provides a contemporary understanding of left ventricular noncompaction cardiomyopathy (LVNC). The disease is increasingly diagnosed in clinical practice, but many clinicians are not familiar with the diagnosis or treatment. LVNC represents a unique type of cardiomyopathy that can have associated findings, such as heart failure and significant arrhythmias, which can lead to morbidity and mortality. Our paper provides care providers, and researchers, with a comprehensive description of the disease along with potential strategies for clinical surveillance and management.