Predicting complete heart block after alcohol septal ablation for hypertrophic cardiomyopathy using a risk stratification model and clinical tool.

BACKGROUND: Alcohol septal ablation (ASA) is a proven method of septal reduction for patients with drug refractory, symptomatic hypertrophic obstructive cardiomyopathy (HOCM). This procedure is associated with a 6.5-11% risk of complete heart block (CHB). OBJECTIVE: The aim of this study is to determine factors that predict CHB and to develop a clinical tool for risk stratification of patients. METHODS: Patients were enrolled into an ongoing ASA study. A total of 636 patient procedures were included, 527 of whom were used in the development of the prediction tool, and 109 of whom were used for independent validation. Multivariate analysis was performed with odds ratios used to develop the clinical prediction tool. This was then internally and externally validated. RESULTS: Of the 527 in the prediction cohort, 46 developed CHB. The predictors of CHB were age ≥50 years, pre-ASA left bundle branch block (LBBB), transient procedural high-grade block, post-ASA PR prolongation ≥68 ms, and new bifascicular block. An 11-point clinical prediction tool was developed to classify these factors. Internal validation using a receiver operating characteristic curve revealed an area under the curve of 0.88 for the clinical prediction tool. External validation using 109 contemporary patients revealed a 98% negative predictive value, 24% positive predictive value, 75% sensitivity, and 81% specificity in high-risk patients. CONCLUSION: Among patients undergoing ASA, the risk of CHB can be predicted with easily obtained clinical and electrocardiographic factors. This clinical prediction tool allows identification of high-risk patients who may benefit from additional monitoring and therapy.

Transaxillary Intra-Aortic Balloon Pump Migration Minimized With a Long Introducer Sheath.

Intra-aortic balloon pump (IABP) has been used more recently as a bridge to cardiac transplantation in hospitalized patients. Femoral IABP limits mobility and rehabilitation; thus, transaxillary approach has been described. However, a transaxillary IABP may migrate, causing significant vascular injury, potential death, or disqualification from transplantation. We describe a case of a 67-year-old male with transaxillary IABP inserted to allow for pre-transplant physical rehabilitation. Due to the unfolded nature of his aorta, the IABP coiled onto itself and migrated several times. We employed a novel technique to mitigate IABP migration using a long 25-cm introducer sheath.

Relation of Lymphangiogenic Factor Vascular Endothelial Growth Factor-D to Elevated Pulmonary Artery Wedge Pressure.

Lymphatic flow is augmented in states of chronic heart failure (cHF). However, the biological mechanism driving increased lymphatic flow capacity (lymphangiogenesis) in cHF is unknown. Recent studies have indicated that vascular endothelial growth factors (VEGF-A, -C, and -D) are involved in lymphangiogenesis. This study examined the association between VEGF-A, -C, and -D levels, invasively measured hemodynamics, and heart failure symptoms. Subjects who underwent clinically indicated right heart catheterization at Medical University of South Carolina between 12/2016 and 7/2018 were eligible for inclusion. These subjects underwent clinical assessment of cHF severity (including 6MWT and KCCQ), hemodynamic assessment with right heart catheterization, laboratory studies including B-type natriuretic peptide, and concomitant measurement of VEGF-A, -C, and -D. Fifty-six patients were included for analysis. Subjects with elevated pulmonary artery wedge pressure (PAWP) had significantly higher VEGF-D levels (263 ± 415 pg/ml vs 65 ± 101 pg/ml; p = 0.02). PAWP was not associated with VEGF-A or VEGF-C levels. When stratified by VEGF-D, subjects with elevated VEGF-D had clinical and hemodynamic characteristics associated with worse HF severity (lower ejection fraction, higher b-type natriuretic peptide, higher PAWP, lower cardiac output), but were not more symptomatic by Kansas City Cardiomyopathy Questionnaire scores and had similar 6-minute walk test distance compared with subjects with lower VEGF-D. Subjects with an elevated VEGF-D were more likely to have a diagnosis of heart failure for >3 years. In conclusion, VEGF-D is associated with elevated PAWP in cHF, and elevated VEGF-D may mitigate cHF symptoms.

Novel Technique of Surgical Management of Scimitar Syndrome.

Scimitar syndrome is a rare congenital abnormality resulting from right-sided pulmonary venous return to the inferior vena cava rather than to the left atrium. It is usually detected in early childhood with symptoms of recurrent chest infection and finding of pulmonary hypertension due to left to right shunt. We report a case of a 40-year-old woman with scimitar syndrome discovered on chest X-ray during evaluation of recurrent pneumonia. Surgical correction was achieved with a novel technique of using a synthetic graft connecting the scimitar vein across the right atrium to the left atrium along with ligation of the scimitar vein connection to the inferior vena cava. The patient continues to do well 10 years after surgery, and the shunt graft shows good flow on echocardiogram. We present her clinical and imaging data and details of the surgical technique along with a brief review of surgical literature.

Percutaneous Coronary Intervention in an 8-Month-Old Infant for Ostial Stenosis of a Reimplanted Left Main Coronary Artery.

Percutaneous coronary intervention (PCI) is a routine procedure undertaken in adult patients. In children, the procedure remains rare and challenging due to a multitude of factors including but not limited to complex congenital heart disease anatomy, catheter and stent to patient size mismatch, and lack of data for post-PCI antiplatelet therapy. We present a case of PCI in an 8-month-old infant with anomalous left coronary artery from pulmonary artery (ALCAPA) who developed severe ostial kinking of the left main coronary artery (LMCA) after surgical reimplantation of the anomalous coronary. A 3.5 × 8 mm Vision bare metal stent was successfully placed into the LMCA and postdilated with excellent results. Follow-up echocardiography at 6 months post-PCI demonstrated a patent stent with normal Doppler flow signals. Despite initial success, the infant developed severe heart failure and was listed for orthotopic heart transplantation at age 20 months, one year after PCI. Given the paucity of published data regarding PCI and outcomes in infants with ALCAPA after surgical reimplantation, we describe our case and present a review of the available literature.

Diagnostic yield and accuracy of coronary CT angiography after abnormal nuclear myocardial perfusion imaging.

We aimed to determine the diagnostic yield and accuracy of coronary CT angiography (CCTA) in patients referred for invasive coronary angiography (ICA) based on clinical concern for coronary artery disease (CAD) and an abnormal nuclear stress myocardial perfusion imaging (MPI) study. We enrolled 100 patients (84 male, mean age 59.6 ± 8.9 years) with an abnormal MPI study and subsequent referral for ICA. Each patient underwent CCTA prior to ICA. We analyzed the prevalence of potentially obstructive CAD (≥50% stenosis) on CCTA and calculated the diagnostic accuracy of ≥50% stenosis on CCTA for the detection of clinically significant CAD on ICA (defined as any ≥70% stenosis or ≥50% left main stenosis). On CCTA, 54 patients had at least one ≥50% stenosis. With ICA, 45 patients demonstrated clinically significant CAD. A positive CCTA had 100% sensitivity and 84% specificity with a 100% negative predictive value and 83% positive predictive value for clinically significant CAD on a per patient basis in MPI positive symptomatic patients. In conclusion, almost half (48%) of patients with suspected CAD and an abnormal MPI study demonstrate no obstructive CAD on CCTA.

Left ventricular end-diastolic pressure affects measurement of fractional flow reserve.

BACKGROUND: Fractional flow reserve (FFR), the hyperemic ratio of distal (Pd) to proximal (Pa) coronary pressure, is used to identify the need for coronary revascularization. Changes in left ventricular end-diastolic pressure (LVEDP) might affect measurements of FFR. METHODS AND MATERIALS: LVEDP was recorded simultaneously with Pd and Pa during conventional FFR measurement as well as during additional infusion of nitroprusside. The relationship between LVEDP, Pa, and FFR was assessed using linear mixed models. RESULTS: Prospectively collected data for 528 cardiac cycles from 20 coronary arteries in 17 patients were analyzed. Baseline median Pa, Pd, FFR, and LVEDP were 73 mmHg, 49 mmHg, 0.69, and 18 mmHg, respectively. FFR<0.80 was present in 14 arteries (70%). With nitroprusside median Pa, Pd, FFR, and LVEDP were 61 mmHg, 42 mmHg, 0.68, and 12 mmHg, respectively. In a multivariable model for the entire population LVEDP was positively associated with FFR such that FFR increased by 0.008 for every 1-mmHg increase in LVEDP (beta=0.008; P<0.001), an association that was greater in obstructed arteries with FFR<0.80 (beta=0.01; P<0.001). Pa did not directly affect FFR in the multivariable model, but an interaction between LVEDP and Pa determined that LVEDP's effect on FFR is greater at lower Pa. CONCLUSIONS: LVEDP was positively associated with FFR. The association was greater in obstructive disease (FFR<0.80) and at lower Pa. These findings have implications for the use of FFR to guide revascularization in patients with heart failure. SUMMARY FOR ANNOTATED TABLE OF CONTENTS: The impact of left ventricular diastolic pressure on measurement of fractional flow reserve (FFR) is not well described. We present a hemodynamic study of the issue, concluding that increasing left ventricular diastolic pressure can increase measurements of FFR, particularly in patients with FFR<0.80 and lower blood pressure.

Alcohol septal ablation for obstructive hypertrophic cardiomyopathy: outcomes in young, middle-aged, and elderly patients.

OBJECTIVES: We compared the efficacy and safety of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) in young, middle-aged, and elderly patients. BACKGROUND: Intersociety guidelines suggest based on limited evidence that young patients with medically refractory symptoms of obstructive HCM should undergo surgical myectomy while elderly patients may be more appropriate for ASA. METHODS: Data for 360 patients undergoing 389 ASAs were prospectively collected and retrospectively analyzed according to age. RESULTS: Young (<45 years), middle-aged (45-64 years), and elderly (≥65 years) patients comprised 28, 40, and 32% of the study population, respectively. Young patients had thicker left ventricular septal walls at baseline, and elderly patients had more comorbidity and dyspnea. Resting, mean left ventricular outflow tract gradients (LVOTGs) were similar across the age groups at baseline (62, 66, and 68 mm Hg, respectively; P = NS for all comparisons). LVOTGs and dyspnea were significantly and similarly improved in all age groups immediately after ASA and through 12 months of follow-up (P < 0.001 for before and after comparisons; P = NS for intergroup comparisons). Complication rates were similar for young and middle-aged patients but higher for elderly patients (9.1 and 6.3% vs. 20.8%, respectively; P ≤ 0.016 for elderly vs. others). Mortality rates for young and middle-aged patients were lower than for elderly patients, but the differences were not statistically significant. CONCLUSIONS: Patients undergoing ASA had significant and similar improvements in LVOTGs and symptoms regardless of age. Procedural complications were increased in elderly patients, who had numerically but not statistically significantly higher mortality rates.

Defibrillation thresholds in hypertrophic cardiomyopathy.

BACKGROUND: Defibrillation threshold (DFT) testing is performed in part to ensure an adequate safety margin for the termination of spontaneous ventricular arrhythmias. Left ventricular mass is a predictor of high DFTs, so patients with hypertrophic cardiomyopathy (HCM) are often considered to be at risk for increased defibrillation energy requirements. However, there are little prospective data addressing this issue. OBJECTIVE: To assess DFTs in patients with HCM and evaluate the clinical predictors of elevated DFTs. METHODS: Eighty-nine consecutive patients with HCM and 600 control patients with ischemic or nonischemic cardiomyopathy underwent a uniform modified step-down DFT testing protocol. DFT was compared between the control and HCM populations. Predictors of elevated DFT were evaluated in the HCM group. RESULTS: There was no difference in DFT between HCM and control groups (10.4 ± 5.8 J vs 11.2 ± 5.6 J, respectively). Among patients with HCM, clinical parameters such as left ventricular ejection fraction, interventricular septal thickness, left ventricular mass, and QRS duration were not predictive of an elevated DFT. Only 3 patients (3.4%) with HCM had a DFT >20 J. CONCLUSION: Patients with HCM do not have elevated DFTs as compared to more typical populations undergoing implantable cardioverter-defibrillator implant; high-energy devices or complex lead systems are not needed routinely in this population.

Implantable cardioverter-defibrillator therapy for primary prevention of sudden death after alcohol septal ablation of hypertrophic cardiomyopathy.

OBJECTIVES: The purpose of this study was to examine the effects of alcohol septal ablation (ASA) on ventricular arrhythmias among patients with obstructive hypertrophic cardiomyopathy (HCM), as measured by appropriate implantable cardioverter-defibrillator (ICD) discharges. BACKGROUND: Alcohol septal ablation is an effective therapy for patients with symptomatic HCM. However, concern has been raised that ASA may be proarrhythmic secondary to the iatrogenic scar created during the procedure. The impact of ASA on ventricular arrhythmias has not been well described. METHODS: This prospective study included 123 consecutive patients with obstructive HCM who underwent ASA and had an ICD implanted for primary prevention of sudden cardiac death (SCD). The ICDs were implanted based on commonly accepted risk factors for SCD in the HCM population. Data from ICD interrogations during routine follow-up were collected. RESULTS: Nine appropriate ICD shocks were recorded over a mean follow-up of 2.9 years in the cohort, which had a mean of 1.5 +/- 0.9 risk factors for SCD. Using Kaplan-Meier survival analysis, the estimated annual event rate was 2.8% over 3-year follow-up. There were no significant differences in the incidence of risk factors between patients who did and did not receive appropriate shocks. CONCLUSIONS: The annual rate of appropriate ICD discharges after ASA is low and less than that reported previously for primary prevention of SCD in HCM. This suggests that ASA is not proarrhythmic. Traditional SCD risk factors did not predict ICD shocks in this cohort.

Follow-up of alcohol septal ablation for symptomatic hypertrophic obstructive cardiomyopathy the Baylor and Medical University of South Carolina experience 1996 to 2007.

OBJECTIVES: This study sought to determine the long-term outcome of alcohol septal ablation (ASA). BACKGROUND: There are inadequate data on the long-term outcome of ASA for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). METHODS: Six hundred and twenty-nine patients were enrolled consecutively (1996 to 2007) and 98.4% (n = 619) underwent ASA with 92% follow-up in 2007. Evaluation included deaths, procedural complications, pacemaker requirement, repeat ASA, and myectomy/valve surgery. Follow-up parameters included angina (Canadian Cardiovascular Society score), dyspnea (New York Heart Association functional class), exercise time, and echocardiographic indices (septal thickness, ejection fraction, resting and provoked gradients). RESULTS: Ethanol (2.6 +/- 1.0 ml) was injected into 1.3 +/- 0.5 septal arteries, inducing a septal infarct. Complications included death 1% (n = 6), permanent pacemaker requirement 8.2% (n = 52), coronary dissection 1.3% (n = 8), and worsening mitral regurgitation 0.3% (n = 2). The mean follow-up was 4.6 +/- 2.5 years (range: 3 months to 10.2 years). During follow-up, New York Heart Association functional class decreased from 2.8 +/- 0.6 to 1.2 +/- 0.5 (p < 0.001); Canadian Cardiovascular Society angina score decreased from 2.1 +/- 0.9 to 1.0 +/- 0 (p < 0.001); and exercise time increased from 4.8 +/- 3.3 to 8.2 +/- 1.0 (p < 0.001) min. The resting and provoked left ventricular outflow tract gradients decreased progressively (p < 0.001) and remained low during follow-up. The septal thickness decreased from 2.1 +/- 0.5 cm to 1.0 +/- 0.1 cm (p < 0.001) and the ejection fraction decreased from 68 +/- 9% to 62 +/- 3% (p < 0.001). The survival estimates at 1, 5, and 8 years were 97%, 92%, and 89%, respectively. CONCLUSIONS: The initial benefits of ASA were maintained during follow-up.

A prospective follow-up of alcohol septal ablation for symptomatic hypertrophic obstructive cardiomyopathy--the Baylor experience (1996-2002).

BACKGROUND: There is a paucity of data on the long-term outcome of alcohol septal ablation (ASA) for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). HYPOTHESIS: The study was undertaken to evaluate the longer-term outcome of ASA therapy for symptomatic HOCM. METHODS: In all, 137 patients were enrolled consecutively (1996-1999) and 130 (95%) (74 men, 56 women, aged 51 +/- 17 years) underwent ASA and had serial prospective follow-up for up to 5 years (mean follow-up 3.6 +/- 1.4 years). Evaluation included angina (Canadian Cardiovascular Society [CCS] score), dyspnea (New York Heart Association [NYHA] class), duration of exercise on treadmill, and echocardiographic indices. RESULTS: Ethanol (3.5 +/- 1.5 cc), injected into 1.5 +/- 0.6 arteries, induced a mean peak plasma creatine kinase (CK) of 1676 +/- 944 units. Complications of procedures included death 1.5% (2/130), heart block requiring permanent pacemaker 13% (17/130), and coronary dissection 4.4% (6/130). Baseline versus last follow-up visit: NYHA class decreased from 3.0 +/- 0.4 to 1.2 +/- 0.6 (p < 0.01); CCS angina score from 2.0 +/- 0.8 to 0.08 +/- 0.4 (p < 0.01); and duration of exercise increased from 322 +/- 207 to 443 +/- 200 s (p < 0.01). Resting left ventricular outflow tract gradient at baseline versus last follow-up visit showed a decrease from 74 +/- 30 to 4 +/- 13 (p < 0.01), and the dobutamine-provoked gradient of 88 +/- 29 decreased to 21 +/- 21 (p < 0.01) mmHg. All-cause mortality over the duration of follow-up was 7.7% (10) giving an annual rate of 2.1%, and cardiac mortality was 2.3% (3) reflecting an annual rate of 0.6%. CONCLUSIONS: Alcohol septal ablation decreased symptoms and improved exercise performance, indicating that it is an effective procedure for symptomatic HOCM.