A case report of percutaneous MitraClip implantation in an adult with a double-outlet right ventricle.

BackgroundAtrioventricular valve regurgitation (AVVR) is present in up to 75% of Fontan patients, and it is associated with an increased risk of Fontan circulation failure, morbidity, and mortality. Traditional treatment options include surgical repair vs. surgical replacement. We present, to the best of our knowledge, one of the first cases of successful trans-catheter repair of severe common AVVR using the MitraClip device.Case summaryA 20-year-old male with a history of double-outlet right ventricle (DORV) with unbalanced common atrioventricular canal to the right ventricle, severely hypoplastic left ventricle, and total anomalous pulmonary venous return status post-Fontan procedure presented with progressively worsening dyspnoea on exertion. Transoesophageal echocardiogram demonstrated severe common AVVR. After discussion of the case during the adult congenital heart disease multidisciplinary conference, patient underwent successful placement of two MitraClip devices, reducing the regurgitation from torrential to moderate.DiscussionMitraClip therapy can be used to alleviate symptoms in patients deemed as high risk for surgery. However, careful attention must be paid to haemodynamics before and after clip placement, which may predict short-term clinical outcomes

The Fontan Circulation: Contemporary Review of Ongoing Challenges and Management Strategies

Since its original description, the Fontan operation has been widely used for the palliation of children with single ventricle physiology and has resulted in an increasing number of these patients surviving to adulthood. The Fontan operation is a unique approach to create a circulation in series without two distinct pumping chambers. Although the Fontan operation increased the survival rates of patients with single ventricle physiology, it carries an inevitable risk of long-term morbidities that impacts the outcomes and quality of life in these patients. In this review, we discuss the challenges resulting from the unique pathophysiology of Fontan circulation and propose management strategies

Safety of SGLT-2 inhibitors in the management of heart failure in the adult congenital heart disease patient population

Background: Sodium glucose transporter 2 inhibitors (SGLT-2i) have shown safety and efficacy in patients with heart failure (HF). However, evidence for the use of SGLT-2i in adult congenital heart disease (ACHD) patients with HF is limited. Methods: We performed a retrospective, single center analysis of 18 patients (>18 years of age) with ACHD and a diagnosis of HF who were initiated on an SGLT-2i. Patient characteristics, including vital signs, laboratory values, concomitant medications, clinical outcomes, and echocardiograms, were obtained as part of standardized clinical care at our ACHD program before and 2–6 months after initiation of SGLT-2i. The primary outcome was to demonstrate safety of SGLT-2i initiation via potential changes in systolic blood pressure, serum sodium, and serum creatinine. Results: Of the 18 patients, 11 (61%) had moderate complexity congenital heart disease while 7 (39%) had great complexity congenital heart disease. Post initiation, there were no significant differences in systolic blood pressure (121.8 ± 20.8 mmHg to 114.4 ± 14.9 mmHg, p = 0.06), sodium level (138.7 ± 2.9 mMol/L to 138.0 ± 2.2 mMol/L, p = 0.75), and creatinine level (0.85 ± 0.18 mg/dL to 0.89 ± 0.18 mg/dL, p = 0.07). There was a statistically significant decline in weight (78.9 ± 22.9 kg to 76.0 ± 23.0 kg, p = 0.0039) but without a statistically significant change in NT-pro NBP (1358.2 ± 2735.0 pg/mL to 601.6 ± 786.1 pg/mL, p = 0.36). Conclusions: We demonstrated the use of SGLT-2i in a small cohort of ACHD population, including patients with complex congenital heart disease, appears safe and well tolerated. The safety and potential efficacy of SGLT-2i in patients with ACHD will require further evaluation in prospective multicenter studies

Computed tomography guided sizing for transcatheter pulmonary valve replacement.

ObjectiveTo evaluate the predictive value of Computed Tomography Angiography (CTA) measurements of the RVOT for transcatheter valve sizing.BackgroundTranscatheter pulmonary valve replacement (TPVR) provides an alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction. We studied 18 patients who underwent catheterization for potential TPVR to determine whether CT imaging can be used to accurately predict implant size.MethodsCases were grouped by RVOT characteristics: native or transannular patch (n = 8), conduit (n = 5) or bioprosthetic valve (n = 5). TPVR was undertaken in 14/18 cases, after balloon-sizing was used to confirm suitability and select implant size. Retrospective CT measurements of the RVOT (circumference-derived (Dcirc) and area-derived (Darea) diameters) were obtained at the level of the annulus, bioprosthesis or conduit. Using manufacturer sizing guidance, a valve size was generated and a predicted valve category assigned: (1) <18 mm, (2) 18-20 mm, (3) 22-23 mm, (4) 26-29 mm and (5) >29 mm. Predicted and implanted valves were compared for inter-rater agreement using Cohen's kappa coefficient.ResultsThe median age of patients was 37 years old (IQR: 30-49); 55% were male. Diagnoses included: Tetralogy of Fallot (12/18), d-Transposition repair (3/18), congenital pulmonary stenosis (2/18) and carcinoid heart disease (1/18). Measurements of Darea (κ = 0.697, p < 0.01) and Dcirc (κ = 0.540, p < 0.01) were good predictors of implanted valve size. When patients with RVOT conduits were excluded, the predictive accuracy improved for Darea (κ = 0.882, p < 0.01) and Dcirc (κ = 0.882, p < 0.01).ConclusionsCT measurement of the RVOT, using Darea or Dcirc, can predict prosthetic valve sizing in TPVR. These measurements are less predictive in patients with conduits, compared to those with a native RVOT or pulmonic bioprosthesis.Condensed abstractWe studied 18 patients who underwent catheterization for TPVR to determine whether CT imaging could be used to accurately predict implant size. Retrospective RVOT measurements were used to generate a predicted valve size, which was compared with implanted valve size for inter-rater agreement. Measurements of Darea (κ = 0.697, p < 0.01) and Dcirc (κ = 0.540, p < 0.01) were good predictors of implanted valve size. When cases with RVOT conduits were excluded, the predictive accuracy improved for Darea (κ = 0.882, p < 0.01) and Dcirc (κ = 0.882, p < 0.01). CT measurement of the RVOT can accurately predict prosthetic valve sizing in TPVR. These measurements are less predictive in patients with conduits

Computed tomography guided sizing for transcatheter pulmonary valve replacement.

ObjectiveTo evaluate the predictive value of Computed Tomography Angiography (CTA) measurements of the RVOT for transcatheter valve sizing.BackgroundTranscatheter pulmonary valve replacement (TPVR) provides an alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction. We studied 18 patients who underwent catheterization for potential TPVR to determine whether CT imaging can be used to accurately predict implant size.MethodsCases were grouped by RVOT characteristics: native or transannular patch (n = 8), conduit (n = 5) or bioprosthetic valve (n = 5). TPVR was undertaken in 14/18 cases, after balloon-sizing was used to confirm suitability and select implant size. Retrospective CT measurements of the RVOT (circumference-derived (Dcirc) and area-derived (Darea) diameters) were obtained at the level of the annulus, bioprosthesis or conduit. Using manufacturer sizing guidance, a valve size was generated and a predicted valve category assigned: (1) <18 mm, (2) 18-20 mm, (3) 22-23 mm, (4) 26-29 mm and (5) >29 mm. Predicted and implanted valves were compared for inter-rater agreement using Cohen's kappa coefficient.ResultsThe median age of patients was 37 years old (IQR: 30-49); 55% were male. Diagnoses included: Tetralogy of Fallot (12/18), d-Transposition repair (3/18), congenital pulmonary stenosis (2/18) and carcinoid heart disease (1/18). Measurements of Darea (κ = 0.697, p < 0.01) and Dcirc (κ = 0.540, p < 0.01) were good predictors of implanted valve size. When patients with RVOT conduits were excluded, the predictive accuracy improved for Darea (κ = 0.882, p < 0.01) and Dcirc (κ = 0.882, p < 0.01).ConclusionsCT measurement of the RVOT, using Darea or Dcirc, can predict prosthetic valve sizing in TPVR. These measurements are less predictive in patients with conduits, compared to those with a native RVOT or pulmonic bioprosthesis.Condensed abstractWe studied 18 patients who underwent catheterization for TPVR to determine whether CT imaging could be used to accurately predict implant size. Retrospective RVOT measurements were used to generate a predicted valve size, which was compared with implanted valve size for inter-rater agreement. Measurements of Darea (κ = 0.697, p < 0.01) and Dcirc (κ = 0.540, p < 0.01) were good predictors of implanted valve size. When cases with RVOT conduits were excluded, the predictive accuracy improved for Darea (κ = 0.882, p < 0.01) and Dcirc (κ = 0.882, p < 0.01). CT measurement of the RVOT can accurately predict prosthetic valve sizing in TPVR. These measurements are less predictive in patients with conduits

Clinical Significance of Coronary Arterial Dominance: A Review of the Literature.

Coronary dominance describes the anatomic variation of coronary arterial supply, notably as it relates to perfusion of the inferior cardiac territories. Differences in the development and outcome in select disease states between coronary dominance patterns are increasingly recognized. In particular, observational studies have identified higher prevalence of poor outcomes in left coronary dominance in the setting of ischemic, conduction, and valvular disease. In this qualitative literature review, we summarize anatomic, physiologic, and clinical implications of differences in coronary dominance to highlight current understanding and gaps in the literature that should warrant further studies