Immediate results of primary balloon dilation for congenital aortic valve stenosis predict the mid-term outcome.

BACKGROUND: Balloon valvuloplasty is the primary treatment for congenital aortic valve stenosis in our centre. We sought to determine independent predictors of reintervention (surgical repair or repeated balloon dilation) after primary valvuloplasty. METHODS: We retrospectively studied patients with congenital aortic valve stenosis who underwent balloon valvuloplasty during 2004-2018. The following risk factors were analysed: aortic valve insufficiency after balloon valvuloplasty >+1/4, post-procedural gradient across the aortic valve ≥35 mmHg, pre-interventional gradient across the valve, annulus size, use of rapid pacing, and balloon/annulus ratio. Primary outcome was aortic valve reintervention. RESULTS: In total, 99 patients (median age 4 years, range 1 day to 26 years) underwent balloon valvuloplasty for congenital aortic valve stenosis. After a mean follow-up of 4.0 years, 30% had reintervention. Adjusted risks for reintervention were significantly increased in patients with post-procedural aortic insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg (HR 2.55, 95% CI 1.13-5.75, p = 0.024). Pre-interventional gradient, annulus size, rapid pacing, and balloon/annulus ratio were not associated with outcome. CONCLUSION: Post-procedural aortic valve insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg in patients undergoing balloon valvuloplasty for congenital aortic valve stenosis confers an increased risk for reintervention in mid-term follow-up.

Mitral Annular Disjunction: Pathophysiology, Pro-Arrhythmic Profile and Repair Pearls.

Mitral annular disjunction (MAD) is a structural abnormality defined by a distinct separation of the mitral valve annulus-left atrial wall continuum and the basal aspect of the posterolateral left ventricle. This anomaly is often observed in patients with myxomatous mitral valve prolapse. Importantly, MAD has been strongly associated with serious ventricular arrhythmias and predisposes to sudden cardiac death. Therefore, we have to emphasize the need to diagnose this morphologic and functional abnormality in routine practice in order to facilitate optimal mitral valve repair and minimize patient risks. Nevertheless, clinical knowledge regarding MAD still remains limited. In the present review, we aim to shed light on several aspects of MAD, including distinct anatomical and pathophysiological characteristics, imaging modalities, association with ventricular arrhythmias, and current methods of treatment.

Relationship of Endothelial Shear Stress with Plaque Features with Coronary CT Angiography and Vasodilating Capability with PET.

Background Advances in three-dimensional reconstruction techniques and computational fluid dynamics of coronary CT angiography (CCTA) data sets make feasible evaluation of endothelial shear stress (ESS) in the vessel wall. Purpose To investigate the relationship between CCTA-derived computational fluid dynamics metrics, anatomic and morphologic characteristics of coronary lesions, and their comparative performance in predicting impaired coronary vasodilating capability assessed by using PET myocardial perfusion imaging (MPI). Materials and Methods In this retrospective study, conducted between October 2019 and September 2020, coronary vessels in patients with stable chest pain and with intermediate probability of coronary artery disease who underwent both CCTA and PET MPI with oxygen 15-labeled water or nitrogen 13 ammonia and quantification of myocardial blood flow were analyzed. CCTA images were used in assessing stenosis severity, lesion-specific total plaque volume (PV), noncalcified PV, calcified PV, and plaque phenotype. PET MPI was used in assessing significant coronary stenosis. The predictive performance of the CCTA-derived parameters was evaluated by using area under the receiver operating characteristic curve (AUC) analysis. Results There were 92 coronary vessels evaluated in 53 patients (mean age, 65 years ± 7; 31 men). ESS was higher in lesions with greater than 50% stenosis versus those without significant stenosis (mean, 15.1 Pa ± 30 vs 4.6 Pa ± 4 vs 3.3 Pa ± 3; P = .004). ESS was higher in functionally significant versus nonsignificant lesions (median, 7 Pa [interquartile range, 5-23 Pa] vs 2.6 Pa [interquartile range, 1.8-5 Pa], respectively; P ≤ .001). Adding ESS to stenosis severity improved prediction (change in AUC, 0.10; 95% CI: 0.04, 0.17; P = .002) for functionally significant lesions. Conclusion The combination of endothelial shear stress with coronary CT angiography (CCTA) stenosis severity improved prediction of an abnormal PET myocardial perfusion imaging result versus CCTA stenosis severity alone. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Kusmirek and Wieben in this issue.

Hospitalizations in adult patients with congenital heart disease: an emerging challenge.

The vast majority of patients with congenital heart disease (CHD) survive into adulthood, but many face lifelong complications, which often result in a hospital admission. The increasing number of hospitalizations in adults with CHD (ACHD) poses a significant challenge for healthcare systems globally, especially as heart failure (HF) is becoming increasingly common in this population and is the leading cause of morbidity and mortality. Besides HF, other major contributors to this increase in admission volume are hospitalizations related to mild lesions, comorbidities and pregnancies. Ιn-hospital mortality ranges between 0.8 and 6.1%, while hospitalizations related to HF predict medium-term mortality in ACHD population. Understanding the predictors of hospitalization and in-hospital mortality is, therefore, important for ACHD healthcare providers, who should identify patients at risk that require escalation of treatment and/or close monitoring. This article reviews the available literature on hospitalization patterns in ACHD patients, with a focus on HF-related hospital admissions and specific diagnostic subgroups.

The aberrant subclavian artery: approach to management.

PURPOSE OF REVIEW: Aberrant subclavian artery (ASCA) represents one of the most common congenital vascular anomalies of the aortic arch. The majority of ASCAs are associated with an aneurysm which occurs at their origin from the descending thoracic aorta, namely, the Kommerell's diverticulum. In this review, we discuss recent data with regards to indications of treatment and surgical management of these anatomical variants. RECENT FINDINGS: Various surgical methods have been described for the repair of ASCA and Kommerell's diverticulum. Traditionally, treatment included open surgery; however, recent studies describe a shift of conventional treatment to more hybrid or endovascular approaches. The heterogeneity in the anatomy and presentation of these clinical entities as well as patient-related factors have prevented conduction of randomized trials for the best available modality of treatment. This dearth of literature is well depicted in the current guidelines. SUMMARY: The optimal surgical procedure has to be tailored to every patient according to the presentation, individual anatomy, and patient's risk profile. Conventional surgery for ASCA and Kommerell's diverticulum has acceptable mortality and complication rates, whereas hybrid repairs report encouraging results. Further studies are required to provide sufficient evidence which will formulate a clear frame of treatment indications and optimal surgical methods, as well as evaluate long-term results following hybrid repair.

Permanent pacemaker implantation in pediatric heart transplant recipients: A systematic review and evidence quality assessment.

Bradyarrhythmias are a common complication following pediatric OHT and may require permanent pacemaker implantation (PPM). The purpose of this study was to investigate the incidence, predictors, and outcomes of children undergoing PPM implantation following OHT. A PRISMA-compliant systematic literature review was performed using the PubMed database and the Cochrane Library (end-of-search date: January 27, 2019). The Newcastle-Ottawa scale and the Joanna Briggs Institute tool were used to assess the quality of cohort studies and case reports, respectively. We analyzed data from a total of 11 studies recruiting 7198 pediatric patients who underwent heart transplant. PPM implantation was performed in 1.9% (n = 137/7,198; 95% CI: 1.6-2.2) of the patients. Most patients underwent dual-chamber pacing (46%, 95% CI: 32.6-59.7). Male-to-female ratio was 1.3:1. Mean patient age at the time of OHT was 10.1 ± 6.3. Overall, biatrial anastomosis was used in 62.2% (95% CI: 52.8-70.6) of the patients. The bicaval technique was performed in the remaining 37.8% (95% CI: 29.4-47.1). Sinus node dysfunction was the most frequent indication for PPM implantation (54.4%; 95% CI: 42.6-65.7) followed by AV block (45.6%; 95% CI: 34.3-57.3). The median time interval between OHT and PPM implantation ranged from 17 days to 12.5 years. All-cause mortality was 27.9% (95% CI: 18.6-39.6) during a median follow-up of 5 years. PPM implantation is rarely required after pediatric OHT. The most common indication for pacing is sinus node dysfunction, and patients undergoing biatrial anastomosis may be more likely to require PPM.

Minimally Invasive Surgery vs Device Closure for Atrial Septal Defects: A Systematic Review and Meta-analysis.

Device closure is the first-line treatment for most atrial septal defects (ASDs). Minimally invasive cardiac surgery (MICS) has been found safe and effective for ASD closure with comparable mortality/morbidity and superior cosmetic results compared to conventional median sternotomy. Our goal was to compare percutaneous versus MICS of ASDs. A systematic review was performed using PubMed and the Cochrane Library (end-of-search date on May 22, 2019). Meta-analyses were conducted using fixed and random effects models. In the present systematic review, we analyzed six studies including 1577 patients with ASDs who underwent either MICS (n = 642) or device closure (n = 935). Treatment efficacy was significantly higher in the MICS (99.8%; 95% CI 98.9-99.9) compared to the device closure group (97.3%; 95% CI 95.6-98.2), (OR 0.1; 95% CI 0.02-0.6). Surgical patients experienced significantly more complications (16.2%; 95% CI 13.0-19.9) compared to those that were treated with a percutaneous approach (7.1%; 95% CI 5.0-9.8), (OR 2.0; 95% CI 1.2-3.2). Surgery was associated with significantly longer length of hospital stay (5.6 ± 1.7 days) compared to device closure (1.3 ± 1.4 days), (OR 4.8; 95% CI 1.1-20.5). Residual shunts were more common with the transcatheter (3.9%; 95% CI 2.7-5.5) compared to the surgical approach (0.95%; 95% CI 0.3-2.4), (OR 0.1; 95% CI 0.06-0.5). There was no difference between the two techniques in terms of major bleeding, hematoma formation, transfusion requirements, cardiac tamponade, new-onset atrial fibrillation, permanent pacemaker placement, and reoperation rates. MICS for ASD is a safe procedure and compares favorably to transcatheter closure. Despite longer hospitalization requirements, the MICS approach is feasible irrespective of ASD anatomy and may lead to a more effective and durable repair.

Recurrent coarctation in Williams syndrome: novel approach of drug-eluting stent implantation.

Patients with Williams syndrome often present with abnormalities of the vascular wall of the aorta and/or the pulmonary artery. Surgery may result in restenosis of the affected vessel. Herein, we report a case of an infant with multiple recurrences of aortic coarctation successfully treated with Zotarolimus drug-eluting stent.

Rapid right ventricular pacing for balloon aortic valvuloplasty: expanding its routine use in neonates and infants.

OBJECTIVE: Rapid right ventricular pacing during balloon aortic valvuloplasty is commonly used to achieve balloon stability in children and adults. There is no consensus for the use of the technique in neonates and infants. We sought to review our institutional experience with rapid right ventricular pacing-assisted balloon aortic valvuloplasty across all age groups and evaluate the safety and effectiveness of the technique in the sub-group of neonates and infants <12months. METHODS: Retrospective study between February, 2011 and February, 2020. RESULTS: A total of 37 patients (Group I: 21 neonates/infants <12months and Group II: 16 children 12 months-16 years) were analysed. Catheter-measured left ventricular to aortic gradient reduced from median of 66 mmHg (with a range from 30 to 125 mmHg) to 14 mmHg (with a range from 5 to 44 mmHg) in Group I and 44 mmHg (with a range from 28 to 93 mmHg) to 18 mmHg (with a range from 2 to 65 mmHg) in Group II (p < 0.001). Procedure and fluoroscopy times were identical in the two groups. Balloon:annulus ratio was 0.94 and 0.88 in Groups I and II, respectively. Freedom from reintervention was 100% for Group I at a median time of 3.2 years and 81% at 2.7 years for Group II. Reinterventions in Group II (3/16 pts) were performed predominantly for complex left ventricular outflow tract stenosis. At follow-up echocardiogram, 45% of patients in Group I had no aortic regurgitation, 30% trace-mild, 20% mild-moderate, and 5% moderate aortic regurgitation, whereas in Group II, 50% of patients had no aortic regurgitation, 32% had mild aortic regurgitation, and 18% mild-moderate aortic regurgitation. Unicuspid valves were only encountered in Group 1 (2/21 pts, 10%) and they were predictive of mild-aortic regurgitation during follow-up (p = 0.003). Ventricular fibrillation occurred in three neonates with suspicion of myocardial ischemia on the pre-procedure echocardiogram. All were successfully defibrillated. CONCLUSIONS: Rapid right ventricular pacing can be expanded in neonates and infants to potentially decrease the incidence of aortic regurgitation and reintervention rates, hence avoiding high-risk surgical bail-out procedures for severe aortic regurgitation in the first year of life. Myocardial ischemia may predispose to ventricular dysrhythmias during rapid right ventricular pacing.

Left ventricular non-compaction in a child with bicuspid aortic valve and aortic coarctation.

We present the case of a 3-year-old boy with bicuspid aortic valve, aortic coarctation, and left ventricular non-compaction. The diagnosis was made post-natally with ultrasonography and was verified by cardiac MRI. Aortic coarctation was initially repaired surgically. At age 3 months, recoarctation and heart failure developed. Balloon angioplasty was performed with immediate improvement. At age 3 years, the patient remains asymptomatic and normotensive.

Cardiovascular magnetic resonance catheterization derived pulmonary vascular resistance and medium-term outcomes in congenital heart disease.

BACKGROUND: Selection of patients with congenital heart disease for surgical septation in biventricular repair or surgical palliation in functionally single ventricles requires low pulmonary vascular resistance (PVR). Where there is uncertainty, PVR can be assessed using hybrid cardiovascular magnetic resonance (CMR) and fluoroscopic (X-Ray) guided cardiac catheterizations (XMR). CMR/XMR catheterization is a validated technique for accurate assessment of pulmonary vascular resistance. However, data concerning its application in clinical practice is lacking. METHODS: PVR assessments were performed in 167 studies in 149 congenital heart disease patients by CMR/XMR catheterization. Data was collated on patient demographics, procedural data, complications and outcomes. Institutional ethics approval was obtained. RESULTS: Median age was 3.6 years (6 days-67 years) and weight 13.8 kg (2.3-122 kg). One hundred and eight studies were in biventricular circulations and 59 in functionally single ventricles. Median radiation dose was 0.72 mSv. A baseline Qp:Qs ≤2.75 in biventricular circulations with left-to-right shunts predicted a PVR ≥6 WU x m(2) with 100% sensitivity and 48% specificity. Median follow up until death or last review was 4.2 years (4 days-11 years). Eighty-four patients had a surgical or catheter intervention based on CMR/XMR catheterization findings at a median of 94 days after the study. This included successful biventricular repair at resting PVR values ≤6 WU x m(2) and Fontan completion at ≤4 WU x m(2). CONCLUSION: PVR measured by CMR/XMR catheterization allows accurate stratification for intervention in patients with congenital heart disease in both, biventricular and univentricular circulations.

Cardiac magnetic resonance imaging in a premature baby with interrupted aortic arch and aortopulmonary window.

Aortopulmonary window is a communication between the main pulmonary artery and the ascending aorta in the presence of two separate semilunar valves. The combination of an aortopulmonary window with interrupted aortic arch is rare. We discuss the unique case of an extremely premature infant weighing 1.7 kilograms who underwent cardiovascular magnetic resonance imaging as a pre-operative assessment in a high-field open 1.0 Tesla magnetic resonance imaging system as a one-stop investigation before complete repair.

Percutaneous upsizing of a Blalock-Taussig shunt.

Percutaneous upsizing of surgically placed Blalock-Taussig shunts is an uncommon practice. We report the case of an 8-month-old infant with single-ventricle physiology, who - due to comorbidities - was deemed unsuitable to proceed with Glenn operation. The 3.5-millimetre Blalock-Taussig shunt was stented successfully with a 5-millimetre pre-mounted stent, resulting in an increase in shunt diameter and oxygen saturation by nearly 30% and 10%, respectively.

Noninvasive imaging prior to percutaneous pulmonary valve implantation.

The majority of patients with congenital heart disease (CHD) who have undergone open heart surgery during childhood are possible candidates for additional transcatheter or surgical interventions. One fifth of these conditions usually involve the right ventricular outflow tract (RVOT). Percutaneous pulmonary valve replacement (PPVR) has been widely established as an alternative, less invasive option to surgical pulmonary valve replacement (SPVR). The variability of RVOT anatomy and size, the relative course of the coronary arteries, and the anatomy of the pulmonary artery branches are factors that determine the success of the intervention and the complication rates. Careful and reliable pre-interventional imaging warrants the selection of suitable candidates and minimizes the risk of complications. 2D and 3D fluoroscopy have been extensively used during pre- and peri-interventional assessment. Established imaging techniques such as cardiovascular magnetic resonance (CMR) and computed tomography (CT) and newer techniques such as fusion imaging have proved to be efficient and reliable tools during pre-procedural planning in patients assessed for PPVR.

Aspergillus Endocarditis of Pulmonary Homograft in a Congenital Heart Disease Patient.

Aspergillus endocarditis (AE) is a life-threatening condition with mortality rates approximating 80%. Herein, we describe the case of a 19-year-old patient with congenitally corrected transposition of great arteries, ventricular septal defect, and pulmonary atresia, who underwent seven cardiac surgical procedures in the past. The patient was operated for AE of a previously implanted pulmonary valve homograft associated with septic pulmonary embolism and right heart failure but succumbed to multi-organ failure three months later. To our knowledge, this is one of two reported cases of Aspergillus infection of a pulmonary homograft, indicating the rarity of the disease.

Cardiovascular MR dobutamine stress in adult tetralogy of Fallot: disparity between CMR volumetry and flow for cardiovascular function.

PURPOSE: To evaluate the MR agreement of cardiac function parameters between volumetric (cine SSFP) and phase contrast flow (PC-flow) assessment in patients with repaired tetralogy of Fallot (r-TOF) and chronic pulmonary regurgitation (PR) at rest and under dobutamine stress (DS-MR). MATERIALS AND METHODS: We studied 18 patients with r-TOF and severe chronic PR (34 ± 12.7 years, PR fraction([flow]) 44 ± 15%) by cardiac MR at rest, 10 and 20 µg/kg/min of dobutamine. We compared analogous functional parameters by volumetry and PC-flow: (i) Systemic output [left ventricle stroke volume (LV(SV)) versus aortic forward flow (AO(FF))], (ii) Pulmonary output [right ventricle stroke volume (RV(SV)) versus pulmonary forward flow (PA(FF))], (iii) PR volume [(RV(SV)-LV(SV)) versus pulmonary backward flow (PA(BF))], (iv) PR fraction [(RV(SV)-LV(SV)/RV(SV)) versus (PA(BF)/PA(FF))]. RESULTS: We found excellent Bland-Altman agreement (mean difference ± limits of agreement, mL/beat/m(2)) at rest for both the systemic (-0.8 ± 5.7) and pulmonary strokes volumes (-0.1 ± 7.6), which slightly deteriorates during DS-MR. The PR volume showed acceptable agreement at rest (-3.6 ± 15.1), but also further deteriorated during stress (5.4 ± 24). In contrast, the PR fraction showed poor agreement equally at rest (-5.6 ± 22.8) and DS-MR (3.2 ± 19.2). CONCLUSION: In r-TOF with chronic PR, analogous functional parameters should not be used interchangeably between volumetric and PC-flow assessment during DS-MR evaluation.

Transhepatic portal vein access for balloon dilation of right upper pulmonary vein stenosis following infradiaphragmatic total anomalous pulmonary venous drainage repair.

Transhepatic cardiac catheterization is now a well-established alternative when conventional venous routes for cardiac access have failed. Some operators prefer this route even routinely, due to the direct access that it offers to the right atrium and atrial septum. We report the case of a newborn baby previously operated on for infradiaphragmatic total anomalous pulmonary venous drainage who represented with right sided pulmonary vein stenosis. Transhepatic portal vein access, first reported here, gave direct access to the right upper pulmonary vein, through the patent descending vein, after the conventional transfemoral venous and retrograde arterial routes had failed.

In situ endothelialization of intravascular stents from progenitor stem cells coated with nanocomposite and functionalized biomolecules.

Owing to their noninvasive nature, coronary artery stents have become popular demand for patients undergoing percutaneous coronary intervention. Late restenosis, in-stent restenosis, and late thrombosis, all mediated by the denuded endothelium, represent the most recurrent failures of vascular stent induction. Higher patency rates of stents can be achieved by restoring the native internal environment of the vessel-an endothelium monolayer. This active organ inhibits the inflammatory reaction to injury responsible for thrombus and intimal hyperplasia, thereby providing a novel therapeutic option to combat the unacceptably high prevalence of restenosis. As the climax of the nanotechnology era approaches, tissue engineering is being explored by means of exploiting the multipotent abilities of stem cells and their adherence to bioactive surface nanocomposite polymers. The endothelium can be reconstructed from neighboring intact endothelium and adherence of circulating endothelium progenitor cells. The latter takes place via a series of signaling events: mobilization, adhesion, chemoattraction, migration, proliferation, and finally their differentiation in mature endothelial cells. A nanotopography surface can orchestrate endothelium formation, attributable to cellular interactions promoted by its nanosize. This review encompasses the prospect of in situ endothelialization, the mechanisms regulating the process, and the advantages of using a new generation of bioactive nanocomposite materials for coating metal stent scaffolds.

Stenting of modified and classical Blalock-Taussig shunts--lessons learned from seven consecutive cases.

In neonates and infants with a shunt-dependent pulmonary circulation, stenosis of the shunt needs to be treated aggressively to prevent a fatal outcome. We report the technique and outcome in seven consecutive neonates and infants with Blalock-Taussig shunt stenosis, who were treated with intraluminal stents. Stenting of classical or modified Blalock-Taussig shunts is technically feasible and can be used as a rescue procedure. Neointimal dissection is less likely compared with balloon angioplasty of the shunt alone.