Dearani, Joseph A.

AbstractObjectivesTo evaluate the outcomes of transcatheter coronary artery fistula (CAF) closure and to identify anatomic/procedural factors that may impact outcomes.BackgroundDue to the rarity of CAF, reported experience with transcatheter closure remains limited and anatomic and procedural factors that may lead to unsuccessful closure, complications, or recanalization of CAF are unclear.MethodsAll patients who underwent transcatheter CAF closure at Mayo Clinic from 1997 to 2018 were retrospectively reviewed. CAF anatomic characteristics, procedural techniques, and clinical/angiographic outcomes were assessed.ResultsA total of 45 patients underwent transcatheter closure of 56 CAFs. The most commonly used devices were embolization coils in 40 (71.4%) CAFs, vascular occluders in 10 (17.8%), or covered stent in 2 (3.6%). Acute procedural success with no or trivial residual flow occurred in 50 (89.3%) CAFs. Residual flow was small in three (5.4%) and large in three (5.4%). Eight (17.8%) patients had complications, including device migration in three, intracranial hemorrhage from anticoagulation in one, and myocardial infarction (MI) in four. MI was a result of covered stent thrombosis or stagnation of flow after closure of large distal CAF. Twenty‐two patients with 27 CAFs had follow‐up angiography after successful index procedure at median time of 423 (IQ 97‐1348) days. Of these, 23 (85.2%) had no/trace flow and 4 had large flow from recanalization.ConclusionsTranscatheter CAF closure is associated with a favorable acute procedural success and complication rate in selected patients. Procedural success and risk for complication are highly dependent on CAF anatomy and closure technique.

Abstract Background: Patients with AL and congestive heart failure have a median survival of less than one year. Methods: Between 1994 and 2005, 11 patients underwent sequential heart transplant followed by autologous peripheral blood stem cell transplant (SCT) for treatment of AL. Patients had heart dominant AL with minimal/no other organ impairment and no evidence of multiple myeloma. Results: Patients ranged in age from 35 to 63 years (median 54). All had biopsy proven amyloidosis and monoclonal protein detectable in serum (73%) or urine (100%). Free light chain assay was available prior to SCT in 6 and was abnormal in all. All patients presented with NYHA Class III or IV heart failure. All but two patients had no other organ involved at the time of heart transplant. One patient had his heart transplant at another facility and had renal involvement at the time of heart transplant. One patient had peripheral nerve involvement documented prior to heart transplant. Five patients had progression of amyloid to involve other organs by the time of SCT. These included: renal (6), peripheral nerve (2), macroglossia (3), gastrointestinal (2). The median time from diagnosis to heart transplant was 3 months (range 1 to 5). The median time from heart transplant to SCT was 8 months, (range 3 to 24). Conditioning chemotherapy consisted of melphalan 200 mg /m2 (6 patients) or melphalan 140 mg/m2 (5 patients). Two patients died of complications before day +100, (TRM of 18%). Hematologic remissions were seen in 8 and organ responses in 3. Five were unable to be evaluated for organ response due to the heart transplant. Three died from progressive amyloidosis at 66, 56.7, and 55 months following SCT. The median survival is 56.7 months from SCT. Six are alive with a median follow-up of 24 months, range 9 to 90 months. Among the 6 surviving patients, one patient relapsed and one patient has failed to improve, but 4 patients are in continued remission. One patient developed secondary myelodysplastic syndrome. Conclusions: Heart transplant followed by SCT is feasible and offers durable remissions for carefully selected patients with cardiac AL. Figure Figure

Objective This study aimed to identify characteristics, spectrum of tricuspid regurgitation (TR) severity and treatment patterns in patients considered for intervention of severe TR at a tertiary centre. The population being considered for TR intervention is currently not well defined and the role of transcatheter interventions is unclear. Methods The study involved 87 patients with severe TR considered for intervention from 1 March 2016 to 12 November 2018 at Mayo Clinic. Patients receiving medications alone were compared with those receiving intervention to identify patterns in demographics, clinical/echocardiographic associations and survival. Results Mean age was 80±9 (56% female), 93% had atrial fibrillation and 64% had chronic kidney disease ≥3 a. Follow-up was 331±276 days; 95% were symptomatic with 6 min walk distance of 270±110 m. Loop diuretics were used in 93%; aldosterone antagonists in 35%. Mean tricuspid annular plane systolic excursion was 15.6±3.8 mm, effective regurgitant orifice area (EROA) 82±32 mm2 and stroke volume index 39±11 mL/m2; 48% had at least moderate right ventricular (RV) dysfunction, and 75% did not undergo intervention. Patients receiving intervention showed trends towards larger EROA (93±33 vs 75±31 mm2), better right ventricular function and more severe symptoms. Overall group 30-day and 1-year survival were 100% and 76%, respectively. Conclusions Patients with severe TR considered for intervention are commonly elderly with atrial fibrillation, advanced TR and RV dysfunction; 75% were treated with medications alone and not offered intervention. Patients with greater EROA, better RV function and more severe symptoms were more likely to receive intervention. These findings have implications for future trial design.

Background: Aiming at fostering local development of cardiology and cardiovascular surgery centers in developing countries, the nonprofit organization Children’s HeartLink (CHL) encourages centers to participate in the International Quality Improvement Collaborative Database for Congenital Heart Disease (IQIC). The definition of parameters and data to evaluate patient treatment provides an opportunity to improve quality of care, reducing morbidity and mortality. The objective of the study was to analyze the outcomes of the partnership between CHL and IQIC database with a single pediatric cardiology and cardiovascular surgery center for seven years providing continuous follow-up to guide actions aiming at morbidity and mortality reduction in patients with pediatric and congenital heart diseases. Methods: Data were collected from January 2011 to December 2017 independently and with external audits and included preoperative information (demographic data, nutritional status, chromosomal abnormalities), Risk Adjustment for Congenital Heart Surgery (RACHS-1) score, and postoperative information such as infections or complications within the first 30 days or until hospital discharge and/or death. Results: In the preoperative period, there was a trend toward an increase in the number of newborn patients. The postoperative period showed significant surgical procedure variations between groups for RACHS-1 risk category ( P = .003), prevalence of risk categories 2 and 3, and an increase in risk categories 4, 5, and 6, mainly in the last two years. Decreases in surgical site infection ( P = .03), bacterial sepsis, and other infections (both P < .001) were observed. At the 30-day postoperative follow-up, there was a decrease of in-hospital ( P = .16) and 30-day ( P = .14) mortality. Conclusion: The partnership between CHL and this seven-year analysis of IQIC database demonstrated structural and human flaws, whose resolution led to significant decrease in infection and reduction in mortality despite an increase in the complexity of our pediatric and congenital heart disease population.