UNASSIGNED: Percutaneous mitral valve repair (PMVR) has evolved to be a standard procedure in suitable patients with mitral regurgitation (MR) not accessible for open surgery. Here, we analyzed the influence of the number and positioning of the clips implanted during the procedure on MR reduction analyzing also sub-collectives of functional and degenerative MR (DMR).
UNASSIGNED: We included 410 patients with severe MR undergoing PMVR using the MitraClip® System. MR and reduction of MR were analyzed by TEE at the beginning and at the end of the PMVR procedure. To specify the clip localization, we sub-divided segment 2 into 3 sub-segments using the segmental classification of the mitral valve.
UNASSIGNED: We found an enhanced reduction of MR predominantly in DMR patients who received more than one clip. Implantation of only one clip led to a higher MR reduction in patients with functional MR (FMR) in comparison to patients with DMR. No significant differences concerning pressure gradients could be observed in degenerative MR patients regardless of the number of clips implanted. A deterioration of half a grade of the achieved MR reduction was observed 6 months post-PMVR independent of the number of implanted clips with a better stability in FMR patients, who got 3 clips compared to patients with only one clip.
UNASSIGNED: In patients with FMR, after 6 months the reduction of MR was more stable with an increased number of implanted clips, which suggests that this specific patient collective may benefit from a higher number of clips.

Human induced pluripotent stem cell derived cardiomyocytes (hiPSC-CMs) are commonly used to model arrhythmogenic cardiomyopathy (ACM), a heritable cardiac disease characterized by severe ventricular arrhythmias, fibrofatty myocardial replacement and progressive ventricular dysfunction. Although ACM is inherited as an autosomal dominant disease, incomplete penetrance and variable expressivity are extremely common, resulting in different clinical manifestations. Here, we propose hiPSC-CMs as a powerful in vitro model to study incomplete penetrance in ACM. Six hiPSC lines were generated from blood samples of three ACM patients carrying a heterozygous deletion of exon 4 in the PKP2 gene, two asymptomatic (ASY) carriers of the same mutation and one healthy control (CTR), all belonging to the same family. Whole exome sequencing was performed in all family members and hiPSC-CMs were examined by ddPCR, western blot, Wes™ immunoassay system, patch clamp, immunofluorescence and RNASeq. Our results show molecular and functional differences between ACM and ASY hiPSC-CMs, including a higher amount of mutated PKP2 mRNA, a lower expression of the connexin-43 protein, a lower overall density of sodium current, a higher intracellular lipid accumulation and sarcomere disorganization in ACM compared to ASY hiPSC-CMs. Differentially expressed genes were also found, supporting a predisposition for a fatty phenotype in ACM hiPSC-CMs. These data indicate that hiPSC-CMs are a suitable model to study incomplete penetrance in ACM.

Patent foramen ovale (PFO) is a remnant of the fetal circulation that remains in a significant portion of the adult population, predisposing to a higher risk of stroke. This risk is further elevated in the postoperative hypercoagulative period. Here we present a case where a patient underwent a total knee arthroplasty and presented with right-sided hemiparesis on post-operative day 2. Subsequently, the patient underwent percutaneous PFO closure with a 25-mm Amplatzer PFO Occluder (Abbott; Chicago, IL, USA). The patient has not had a stroke since the PFO closure. Recent randomized trials have demonstrated superiority of percutaneous PFO closure over standard-of-care medical therapy for secondary prevention of PFO-associated stroke. Since post-operative PFO-associated stroke is under-recognized in clinical practice, further large-cohort studies are needed to evaluate whether PFO screening and device closure would decrease post-operative stroke risk for noncardiac surgeries.
UNASSIGNED: Patent foramen ovale (PFO) is a remnant of the fetal circulation commonly found in the adult population, which can increase the risk of stroke. Stroke is a complication of PFO, yet closure of this remnant only occurs on a specific case-by-case basis. Further research in this area is required to determine whether a larger population would benefit from PFO closure.

Limited venous access and lateral left ventricular scar are impediments to traditional cardiac resynchronization therapy. We present a case where placement of an implantable cardioverter-defibrillator from a femoral approach while using left bundle branch area pacing led to clinical improvement. (Level of Difficulty: Intermediate.).

We present the case of a 55-year old Caucasian man with Eisenmenger syndrome secondary to uncorrected aorto-pulmonary window, whose clinical course has been complicated by recurrent cerebral abscesses and dynamic tricuspid annular caseation with probable pulmonary embolization. (Level of Difficulty: Intermediate.).

Homocystinuria is a rare genetic disease with autosomal recessive pattern. It is reported to be highest in Arabian descend and could cause thrombosis, but mainly peripherally. Cardiac amorphous tumor has been recognized in the past 20 years and it is also a very rare cause primary benign tumor of the heart. Most of the cases reported to be associated with end-stage renal disease. Homocystinuria associated with Cardiac Amorphous tumor is extremely rare. Up to our knowledge, there has been only one other case has been reported. Our patient is a 14-year-old female known case of homocystinuria presented with dyspnea and leg edema. On workup was found to have a mass in the right atrium extending to superior vena cava and inferior cava. Surgery undertaken on cardiopulmonary bypass partial resection of the mass was done and result came back as cardiac amorphous tumor. We assume the cause of this sinister complication of her primary illness is calcification of thrombus as stated in literature. And also recommend further studies regarding issue on hand.

While in labor, a 37-year-old woman developed acute dyspnea, hypoxemia, and tachycardia. Transthoracic echocardiography demonstrated severe right ventricular dilation and dysfunction, raising the suspicion of acute pulmonary embolism. The patient indeed had bilateral pulmonary embolism, necessitating percutaneous thrombectomy. Her course was complicated by another saddle pulmonary embolus, heparin-induced thrombocytopenia, and COVID-19 infection. This clinical case illustrates the importance of prompt diagnosis of acute pulmonary embolism in a peripartum female patient, the multidisciplinary approach of management, and how to approach clinical complications such as heparin-induced thrombocytopenia. Furthermore, long-term management in acute pulmonary embolism is presented.

Transvenous laser-assisted lead extraction is successful, with a low procedural complication rate for a wide range of indications. Here, we report a case of right internal jugular triple-lumen central venous catheter fracture and subsequent embolism to the right pulmonary artery during laser lead extraction that was successfully retrieved with a gooseneck snare. (Level of Difficulty: Advanced.).

Coronary injury or spasm induced by endocardial radiofrequency catheter ablation in the right ventricle (RV) has been rarely reported. We introduce a case of coronary injury from a young patient who underwent catheter ablation for idiopathic ventricular arrhythmia originating from the RV septum. (Level of Difficulty: Advanced.).

Friedreich Ataxia (FRDA) is an autosomal recessive disease in which a mitochondrial protein, frataxin, is severely decreased in its expression. In addition to progressive ataxia, patients with FRDA often develop a cardiomyopathy that can be hypertrophic. This cardiomyopathy is unlike the sarcomeric hypertrophic cardiomyopathies in that the hypertrophy is associated with massive mitochondrial proliferation within the cardiomyocyte rather than contractile protein overexpression. This is associated with atrial arrhythmias, apoptosis, and fibrosis over time, and patients often develop heart failure leading to premature death. The differences between this mitochondrial cardiomyopathy and the more common contractile protein hypertrophic cardiomyopathies can be a source of misunderstanding in the management of these patients. Although imaging studies have revealed much about the structure and function of the heart in this disease, we still lack an understanding of many important clinical and fundamental molecular events that determine outcome of the heart in FRDA. This review will describe the current basic and clinical understanding of the FRDA heart, and most importantly, identify major gaps in our knowledge that represent new directions and opportunities for research.