Safety and efficacy of stenting for aortic arch hypoplasia in patients with coarctation of the aorta.

BACKGROUND: Despite a successful repair procedure for coarctation of the aorta (CoA), up to two-thirds of patients remain hypertensive. CoA is often seen in combination with abnormal aortic arch anatomy and morphology. This might be a substrate for persistent hypertension. Therefore, we performed endovascular aortic arch stent placement in patients with CoA and concomitant aortic arch hypoplasia or gothic arch morphology. The goal of this retrospective analysis was to investigate the safety and efficacy of aortic arch stenting. METHODS: A retrospective analysis was performed in patients who underwent stenting of the aortic arch at the University Medical Center Utrecht. Measurements collected included office blood pressure, use of antihypertensive medication, invasive peak-to-peak systolic pressure over the arch, and aortic diameters on three-dimensional angiography. Data on follow-up were obtained at the date of most recent outpatient visit. RESULTS: Twelve patients underwent stenting of the aortic arch. Mean follow-up duration was 14 ± 11 months. Mean peak-to-peak gradient across the arch decreased from 39 ± 13 mm Hg to 7 ± 8 mm Hg directly after stenting (p < 0.001). There were no major procedural complications. Mean systolic blood pressure decreased from 145 ± 16 mm Hg at baseline to 128 ± 9 mm Hg at latest follow-up (p = 0.014). CONCLUSION: This retrospective study shows that stenting of the aortic arch is successful when carried out in a state-of-the-art manner. A direct optimal angiographic and haemodynamic result was shown. No major complications occurred during or after the procedure. At short- to medium-term follow-up a decrease in mean systolic blood pressure was observed.

Three-dimensional rotational angiography in children with an aortic coarctation.

BACKGROUND: Children with aortic coarctations (CoA) are increasingly percutaneously treated. Good visualisation of the CoA is mandatory and can be obtained with three-dimensional rotational angiography (3DRA). This study aims to compare the diagnostic and therapeutic additional value of 3DRA with conventional biplane angiography (CA) in children with a CoA. METHODS: Patients undergoing percutaneous treatment of CoA with balloon angioplasty (BA) or stent between 2003 and 2015, were retrospectively reviewed on success rate, complications, radiation and technical settings. Diagnostic quality of CA and 3DRA and additional value of 3DRA were scored. RESULTS: In total, 134 patients underwent 183 catheterisations, 121 CA and 62 3DRA-guided. Median age was 0.52 years in the BA group and 11.19 years in the stent group. 3DRA was superior to CA in displaying the left ventricle (p = 0.008), ascending aorta (p < 0.001), aortic arch (p = 0.005) and coronary arteries (p < 0.001). In the BA group, 3DRA had a significantly higher success rate than CA (100.0 % versus 68.9 %, p = 0.016). All stent interventions were successful. Complication rates did not differ significantly. The median total dose area product did not significantly differ between CA and 3DRA in the BA (27.88 µGym2/kg versus 15.81 µGym2/kg, p = 0.275) or stent group (37.34 µGym2/kg versus 45.24 µGym2/kg, p = 0.090). 3DRA was of additional value in 96.8 % of the interventions. CONCLUSIONS: 3DRA is superior to CA in diagnostic quality and not associated with increased radiation exposure. It provides high additional value in guiding CoA related interventions.

Congenital posterior pole cataract and adult onset dilating cardiomyopathy: expanding the phenotype of αB-crystallinopathies.

Mutations in the αB-crystallin gene (CRYAB) have been reported in desmin-related myopathies, with or without cardiac involvement. Mutations in this gene have also been documented in large multi-generation families with autosomal dominant congenital posterior pole cataract (CPPC). In these congenital cataract families no cardiac or muscular phenotype was reported. This report describes a family with an unusual read-through mutation in CRYAB, leading to the elongation of the normal αB-crystallin protein with 19 amino acid residues. Affected family members combine a CPPC with an adult onset dilated cardiomyopathy (DCM), thereby expanding the αB-crystallinopathy phenotype. Repolarisation abnormalities preceded the onset of cardiomyopathy and were already present in childhood. No skeletal myopathy was observed. This report illustrates that congenital cataract can be a prelude to more severe disease even outside the context of inborn errors of metabolism. The identification of a CRYAB mutation in this family supports the notion that mutations in this gene are a rare cause of genetically determined DCM. The combined congenital cataract/cardiomyopathy phenotype adds to our understanding of the complex phenotypic spectrum of αB-crystallinopathies.

[Accurate assessment of heart murmurs in children: thorough medical history and physical examination required]. / Beoordeling van hartgeruisen bij kinderen: zorgvuldige anamnese en lichamelijk onderzoek vereist.

4 children, boys aged 12, 5, 1.5 and 11 years, had a heart murmur. The 12-year-old boy could also not finish a football match and appeared to have atrioseptal defects (ASD). The 1.5-year-old boy had pulmonary symptoms that were not responsive to asthma medication; he also had ASD. The 11-year-old boy had had chest pain and pressure following exertion for 2 years; he appeared to have an aortic stenosis. Symptoms disappeared in all 3 patients after surgical correction. In the 5-year-old asymptomatic boy the murmur was deemed to be innocent following medical history and physical examination. Children frequently have heart murmurs. Most heart murmurs are innocent but some are caused by heart defects. Careful evaluation of the medical history and physical examination are critical in the differentiation of innocent and pathological heart murmurs. Routine supplementary diagnostic tests in children with heart murmurs are of limited value and are often misleading. One should inquire about specific and nonspecific symptoms and also perform systematic inspection, palpation and auscultation to identify any characteristics that suggest a heart murmur caused by a heart defect.

Contractile adaptations preserving cardiac output predispose the hypertrophied canine heart to delayed afterdepolarization-dependent ventricular arrhythmias.

BACKGROUND: In dogs, chronic complete atrioventricular block (CAVB) results in structural (biventricular hypertrophy) and electrical (delayed repolarization) remodeling, which predisposes the heart to torsade de pointes arrhythmias. We assessed the contractile alterations in the CAVB dog and tested the hypothesis that these adaptations increase delayed afterdepolarization (DAD)-dependent triggered arrhythmias. METHODS AND RESULTS: Steady-state and dynamic (fast pacing: 1 to 68 stimuli) left and right ventricular systolic and diastolic parameters were determined by positive and negative inotropic interventions at acute AVB and CAVB. Concomitantly, left and right ventricular endocardial monophasic action potentials were registered. In CAVB, all systolic contractile parameters were markedly increased, resulting in preserved cardiac output. The increase was most pronounced at low heart rates, altering the force-frequency response. At both acute AVB and CAVB, the degree of potentiation of cardiac function with pacing was dependent on the number of stimuli and showed a maximum at 8 to 13 stimuli. With CAVB, this potentiation curve was shifted upward, and it was only then that pacing resulted in DADs (in 8 of 10 dogs) and ectopic beats (EBs, in 6 of 10 dogs). The incidence of EBs in relation to the number of stimuli also had a maximum at 8 to 13 stimuli. Ouabain increased the incidence of DADs and EBs, whereas the negative inotropic interventions prevented them completely. CONCLUSIONS: The alterations responsible for improvement in systolic contractile function in CAVB dogs predispose the hypertrophied heart to DAD-dependent triggered arrhythmias during positive inotropic interventions.

Chronic amiodarone evokes no torsade de pointes arrhythmias despite QT lengthening in an animal model of acquired long-QT syndrome.

BACKGROUND: Amiodarone is an effective antiarrhythmic drug rarely associated with torsade de pointes arrhythmias (TdP). The noniodinated compound dronedarone could resemble amiodarone and be devoid of the adverse effects. In the dog with chronic complete atrioventricular (AV) block (CAVB) and acquired long-QT syndrome, the electrophysiological and proarrhythmic properties of the drugs were compared after 4 weeks of oral treatment. METHODS AND RESULTS: Amiodarone (n=7, 40 mg. kg(-1). d(-1)) and dronedarone (n=8, 20 mg/kg BID) were started at 6 weeks of CAVB (baseline). Six dogs served as controls. Surface ECGs and endocardially placed monophasic action potential catheters in the left (LV) and right (RV) ventricles were recorded to assess QTc time, action potential duration (APD), interventricular dispersion (DeltaAPD=LV APD minus RV APD), early afterdepolarizations (EADs), ectopic beats, and TdP. Both amiodarone (+21%) and dronedarone (+31%) increased QTc time. Amiodarone showed no increase in DeltaAPD in 4 of 7 dogs, whereas dronedarone augmented DeltaAPD in 7 of 8 animals. After dronedarone, TdP occurred in 4 of 8 dogs with the highest DeltaAPD (105+/-20 ms). TdP was never seen with amiodarone, not even in the dogs that had DeltaAPD values comparable to those with dronedarone. Furthermore, a difference existed in EADs and ectopic activity incidence (dronedarone 3 of 8; amiodarone 0 of 7), which was also seen during an epinephrine challenge. CONCLUSIONS: In the CAVB dog model, both amiodarone and dronedarone prolong QT time (class III effect). The absence of TdP with amiodarone seems to be related to homogeneous APD lengthening in the majority of dogs and the lack of EADs and/or ventricular ectopic beats in all.