Left ventricular restrictive physiology in kids with atrial septal defects: Something unexpected!

Atrial septal defect (ASD) associated with left ventricular (LV) diastolic dysfunction (DD) is rare in children. DD is common in elderly patients undergoing ASD closure. Restrictive physiology among children undergoing percutaneous ASD closure is something unexpected that has not been described before in the literature. Hence, we report a child referred to our center for ASD closure in whom an LV DD has been unmasked with a balloon occlusion test and has been managed with pharmacological preconditioning and subsequent ASD closure with good outcome. Albeit rare, LV DD can occur in children undergoing ASD closure.

Percutaneous Pulmonary Valve Implantation.

Percutaneous pulmonary valve implantation (PPVI) is recognized as a feasible and low risk alternative to surgery to treat dysfunctional right ventricular outflow tract (RVOT) in usually pluri-operated patients. Evolving technology allowed to develop different kind of prosthesis and to go from an initial treatment exclusively of stenotic conduit to an actual approach extended also to wide native RVOT. The Melody transcatheter pulmonary valve (TPV) and the Edwards Sapien valve are nowadays the most commonly implanted prostheses. However, other devices have been developed to treat large RVOT (i.e., the Venus p-valve, the Medtronic Harmony TPV, the Alterra Adaptive Prestent, and the Pulsta valve). Indications for PPVI are the same as for surgical interventions on pulmonary valve, with limits related to the maximum diameter of the available percutaneous prosthesis. Therefore, an accurate preoperative evaluation is of paramount importance to select patients who could benefit from this procedure. The overall periprocedural mortality incidence is around 1.4%, while freedom from RVOT reintervention ranges from 100% at 4 months to 70% at 70 months, according to the different published studies.

Failing mitral homograft in the tricuspid position treated with a percutaneous approach.

: Patients with degenerated mitral homograft in tricuspid position, albeit rare, can nowadays come to our attention. Here we report on a case that shows that the transcatheter approach can be successfully adopted, leading to an evident improvement in the clinical condition, especially in symptomatic patients who cannot undergo surgical intervention.

Techniques, Timing, and Prognosis of Transcatheter Post Myocardial Infarction Ventricular Septal Defect Repair.

PURPOSE OF REVIEW: This review aims to delineate the actual role of percutaneous intervention in the closure of post-myocardial infarction ventricular septal defect (post-MI VSD) and to briefly summarize the main steps of this procedure. RECENT FINDINGS: Most of the published studies report experiences using Amplatzer devices for post-MI VSD closure. In the acute phase, morbidity and mortality are quite high up to 70%, with a mean success rate of 90%, with 95% confidence intervals from 60 to 100%, and a 30-day mortality of 40%, with 95% confidence intervals from 0 to 55%. In the chronic phase, that is 14 days after myocardial infarction, results are very encouraging, with lower morbidity and mortality (23% at 30 days) and a higher rate of complete closure. A multimodality imaging approach has been proposed in order to increase the success rate of this procedure. Percutaneous closure is a safe and effective procedure in highly specialized centers and an appropriate patient selection is of paramount importance to the success of the procedure. Device closure of post-MI VSD can be considered a true alternative to the standard surgical approach. However, many problems still exist for percutaneous post-MI VSD treatment.

Atrial septal defect (ASD) device trans-catheter closure: limitations.

Transcatheter closure is a widespread technique used to treat secundum atrial septal defects (ASDs). When compared to surgery, it provides a less invasive approach with quicker recovery and reduced physical and psychological impact. Nowadays, almost 85-90% of all secundum ASD can be closed by using a transcatheter approach. However, several limitations may have a significant impact on the feasibility and success of percutaneous ASD closure. Limitations can be grouped as: (I) anatomical; (II) device-related; (III) associated defects and natural history associated issues; (IV) physiological; (V) complications. Physician should be aware of potential limits of percutaneous ASD closure.

Management of balloon rupture during a percutaneous pulmonary valve implantation procedure.

Percutaneous pulmonary valve implantation is increasingly adopted as an alternative procedure to surgery in dysfunctional homograft, and in patients with "native" or wide right ventricle outflow tract dysfunction. Pre-stenting is mandatory in this category of patients for many reasons, one of which is to create an adequate landing zone for the bioprosthesis. Here we report on a tricky situation that occurred during pre-stenting, and we describe how we successfully overcame it.

Coronary Sinus Reducer Implantation for the Treatment of Chronic Refractory Angina: A Single-Center Experience.

OBJECTIVES: The aim of this study was to assess the safety and efficacy of the Reducer in a real-world cohort of patients presenting with refractory angina. BACKGROUND: The coronary sinus Reducer is a novel device to aid in the management of patients with severe angina symptoms refractory to optimal medical therapy and not amenable to further revascularization. METHODS: Fifty patients with refractory angina and objective evidence of myocardial ischemia who were judged unsuitable for revascularization were treated with coronary sinus Reducer implantation at a single center between March 2015 and August 2016. Safety endpoints were procedural success and the absence of device-related adverse events. Efficacy endpoints, assessed at 4- and 12-month follow-up, were a reduction in Canadian Cardiovascular Society angina class, improvement in quality of life assessed using the Seattle Angina Questionnaire, improvement in exercise tolerance assessed using the 6-min walk test, and reduction in pharmacological antianginal therapy. RESULTS: Procedural success was achieved in all patients, with no device-related adverse effects during the procedure or at follow-up. Regarding the efficacy endpoint, 40 patients (80%) had at least 1 reduction in Canadian Cardiovascular Society class, and 20 patients (40%) had at least 2 class reductions, with a mean class reduction to 1.67 ± 0.83 vs. 2.98 ± 0.52 (p < 0.001) at 4-month follow-up. All Seattle Angina Questionnaire items improved significantly (p < 0.001 for all). A significant increment in 6-min walk distance to 388.6 ± 119.7 m vs. 287.0 ± 138.9 m (p = 0.004) was observed. Sixteen patients (32%) and 3 patients (6%) demonstrated reductions of at least 1 or 2 antianginal drugs, respectively. The benefit of Reducer implantation observed at 4-month follow-up was maintained at 1 year. CONCLUSIONS: In this real-world, single-center experience, implantation of the coronary sinus Reducer appeared safe and was associated with reduction in anginal symptoms and improvement in quality of life in patients with refractory angina who were not candidates for further revascularization.

Emergency transcatheter closure of a stented PDA in a patient with pulmonary atresia and intact ventricular septum: be ready for the unexpected!

Patients with congenital heart disease and duct-dependent pulmonary circulation can undergo stenting of the patent ductus arteriosus (PDA). This case shows that, due to the physiological changes occurring after stent implantation, sometimes it is necessary to close the stented PDA rather than to redilate it.

Tricuspid regurgitation as a complication of Edwards Sapien XT valve implantation in pulmonary position a problem to deal with.

Nowadays, percutaneous pulmonary valve implantation (PPVI) is a suitable alternative to surgical procedure in patients with right ventricle outflow tract dysfunction. Two valves are currently available for PPVI: the Melody valve and the Edwards Sapien valve. The following complications may occur: coronary artery compression, deformation of the aortic root, stent or valve embolization, damage of distal pulmonary arteries, access site injuries. Here, we report on three cases of severe tricuspid regurgitation due to valvular and subvalvular apparatus damage during Edwards Sapien XT valve implantation.

Heart failure in grown-up congenital heart disease.

The increasing survival to adulthood of patients with congenital heart disease (CHD) has changed the epidemiology of adult CHD (ACHD) patients and has led to an increment in hospitalization rates due to heart failure (HF). ACHD patients hospitalized for HF have a five-fold higher risk of death than those compensated. HF occurs predominantly in patients with tetralogy of Fallot, single ventricles, and after the Mustard operation for transposition of the great arteries. Diagnostic strategies applied in acquired HF patients are usually used to evaluate ACHD patients, but sometimes this can postpone the identification of HF that can become manifest with unusual and peculiar signs or symptoms. In the same way, therapeutic management resembles the acquired HF one, even if no large randomized clinical trials have been conducted in ACHD patients. Therefore, a close monitoring in dedicated units is mandatory in order to identify in time HF manifestations and manage them adequately.

Coronary microvascular dysfunction in chronic inflammatory rheumatoid diseases.

Chronic inflammatory rheumatoid diseases (CIRD) such as rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis are an important risk factor for the development of ischaemic heart disease and a source of high cardiovascular morbidity and mortality. In patients affected by CIRD, inflammation can affect coronary microvascular function and contribute to the development of myocardial ischemia and cardiovascular events, even in the absence of obstructive epicardial coronary artery disease. Understanding the molecular aspects that underlie the development of coronary microvascular dysfunction (CMD) in CIRD is of fundamental importance to identify specific therapeutic targets. In this article, we review the pathogenic mechanisms leading to CMD in CIRD, including the controversial results obtained with the use of different therapeutic strategies. We also propose that a practical diagnostic algorithm as the identification of CMD in patients with CIRD may lead to effective measures to prevent the development of angina pectoris and reduce the risk of rapid disease progression.

Coronary microvascular dysfunction in asymptomatic patients affected by systemic sclerosis - limited vs. diffuse form.

BACKGROUND: This observational study was designed to evaluate the prevalence of coronary microvascular dysfunction (CMD) in asymptomatic patients affected by systemic sclerosis (SSc), stratifying the results according to the limited (lcSSc) and the diffuse (dcSSc) forms of the disease. METHODS AND RESULTS: We enrolled 19 consecutive asymptomatic patients with dcSSc (n=7) or lcSSc (n=12). In all subjects, coronary flow reserve (CFR) was assessed by measuring diastolic coronary flow velocities in the left anterior descending artery by pulsed wave Doppler at baseline and after dipyridamole infusion (0.84 mg·kg(-1)·6 min(-1)). Wall motion score index was evaluated at baseline and during stress. We enrolled 20 healthy subjects as controls. Mean CFR was 1.96±0.62 in patients and 2.69±0.47 in controls (P<0.001). Abnormal values of CFR (≤2) were significantly more prevalent in patients than in controls (10/19 vs. 0/20; P<0.001) and in the dcSSc subgroup than in the lcSSc subgroup (6/7 vs. 4/12; P=0.05). An inverse relationship between disease duration (from time of onset of Raynaud's phenomenon) and CFR value was observed in the lcSSc group (correlation coefficient -0.583; P=0.046). Neither patients nor controls had wall motion abnormalities during dipyridamole administration. CONCLUSIONS: A blunted CFR, most likely because of CMD, is more frequent in patients affected by the dcSSc form in the early stages of the disease, whereas it seems to appear later in lcSSc.

[Cardiac involvement at rest in patients with systemic sclerosis: differences between the limited and the diffuse form of the disease]. / Coinvolgimento cardiaco a riposo in pazienti affetti da sclerosi sistemica: differenze fra forma diffusa e forma limitata.

BACKGROUND: Heart involvement confers a poor prognosis in patients affected by systemic sclerosis (SSc). Nevertheless, the prevalence of heart involvement in these patients is not fully known. In this study we analyzed the most frequent manifestations of heart involvement at rest in a cohort of patients with SSc, comparing their prevalence in patients with the limited form (lcSSc) and in patients with the diffuse form (dcSSc) of the disease, taking also into account the duration of the disease. METHODS: We thoroughly evaluated with electrocardiographic and echocardiographic examinations 174 patients affected by SSc who were followed at our hospital between 2001 and 2011. They were divided according to the disease subtype (dcSSc vs lcSSc) and information about the disease duration was available for 121 of them (calculated from the onset of the Raynaud's phenomenon). RESULTS: Patients with dcSSc had a greater prevalence of heart involvement at rest when evaluated within 5 and 10 years after the onset of the Raynaud's phenomenon (p=0.0051 within 5 years and p=0.035 within 10 years). Indeed, patients with dcSSc had a greater prevalence of atrioventricular conduction abnormalities within 20 years after the onset of the Raynaud's phenomenon (p=0.03 within 10 years and p=0.04 within 20 years) and a greater prevalence of valvular abnormalities within 5 years (p=0.04). In dcSSc there was a greater prevalence of rhythm disturbances in patients with a disease duration ≤20 or >20 years (p=0.04 within 20 years and p=0.04 after 20 years), but not in those with a disease duration ≤5 and ≤10 years. Left ventricular hypertrophy had a greater prevalence in dcSSc after 20 years of disease duration (p=0.02). CONCLUSIONS: Cardiac manifestation occurs earlier and more frequently in patients affected by dcSSc than in patients with lcSSc.