Anti-Kv1.4 Antibody Without Myasthenia Gravis: A Rare Cause of Autoimmune Myocarditis and Myositis.

Anti-Kv1.4 antibody is often detected in thymoma-associated myasthenia gravis patients with anti-acetylcholine receptor antibody. Herein, we describe 2 patients with concurrent myocarditis and myositis. In both cases, anti-Kv1.4 antibody was positive despite the absence of thymoma and anti-acetylcholine receptor antibody, and immunosuppressants eventually resolved their symptoms and cardiac function. (Level of Difficulty: Advanced.).

Decoding Postinfarction Left Ventricular Pseudoaneurysm.

Recognizing true from pseudo left ventricular aneurysm after myocardial infarction is paramount to guide clinical management and determine need for surgical urgency. We discuss a case of a postinfarction pseudoaneurysm that poses unique anatomic challenges and may hold a secret "DaVinci code" beyond current diagnostic criteria. (Level of Difficulty: Advanced.).

Improved mid-term stability of MR reduction with an increased number of clips after percutaneous mitral valve repair in functional MR.

Background: 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). Results: 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. Results: 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. Conclusions: 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.

A Rare Case of Radiation-Associated Aortic and Mitral Valve Stenosis Treated With Transcatheter Valve Replacements.

Radiation therapy is the standard of care for achieving cure for many thoracic malignancies, but it can result in long-term cardiovascular sequelae such as valve disease. We describe a rare case of severe aortic and mitral stenosis due to prior radiation therapy for giant cell tumor treated successfully with percutaneous aortic and off-label mitral valve replacements. (Level of Difficulty: Intermediate.).

Three-dimensional printing and virtual reconstruction in surgical planning of double-outlet right ventricle repair.

Objectives: For more than a decade, 3-dimensional (3D) printing has been identified as an innovative tool for the surgical planning of double-outlet right ventricle (DORV). Nevertheless, lack of evidence concerning its benefits encourages us to identify valuable criteria for future prospective trials. Methods: We conducted a retrospective study involving 10 patients with DORV operated between 2015 and 2019 in our center. During a preoperative multidisciplinary heart team meeting, we harvested surgical decisions following a 3-increment step process: (1) multimodal imaging; (2) 3D virtual valvular reconstruction (3DVVR); and (3) 3D-printed heart model (3DPHM). The primary outcome was the proportion of predicted surgical strategy following each of the 3 steps, compared with the institutional retrospective surgical strategy. The secondary outcome was the change of surgical strategy through 3D modalities compared with multimodal imaging. The incremental benefit of the 3DVVR and 3DPHM over multimodal imaging was then assessed. Results: The operative strategy was predicted in 5 cases after multimodal imaging, in 9 cases after 3DVVR, and the 10 cases after 3DPHM. Compared with multimodal imaging, 3DVVR modified the strategy for 4 cases. One case was correctly predicted only after 3DPHM inspection. Conclusions: 3DVVR and 3DPHM improved multimodal imaging in the surgical planning of patients with DORV. 3DVVR allowed a better appreciation of the relationships between great vessels, valves, and ventricular septal defects. 3DPHM offers a realistic preoperative view at patient scale and enhances the evaluation of outflow tract obstruction. Our retrospective study demonstrates benefits of preoperative 3D modalities and supports future prospective trials to assess their impact on postoperative outcomes.

Cystic Tumor of the Atrioventricular Node Causing Rapid-Onset and Reversible Complete Heart Block.

Cystic tumors of the atrioventricular node are rare primary cardiac neoplasms, frequently reported as a postmortem diagnosis during autopsy. Despite their small size, they can present with complete heart block or sudden cardiac death. Herein, we present a case of rapidly progressing high-grade atrioventricular block that improved after surgery. (Level of Difficulty: Intermediate.).

Early Valve Thrombosis Management After Successful Transcatheter Tricuspid Valve-in-Valve Implantation.

Transcatheter tricuspid valve-in-valve implantation is a critical option in high risk bioprosthetic tricuspid valve dysfunction. In this case report, balloon-expandable transcatheter heart valve was implanted successfully into the tricuspid valve and early thrombosis was managed successfully. (Level of Difficulty: Intermediate.).

Asymptomatic Apical Hypertrophic Cardiomyopathy in an Elite Competitive Athlete.

A 17-year-old male elite athlete presented for evaluation after an abnormal pre-competitive college screening electrocardiogram. Subsequent evaluation revealed the presence of hypertrophic cardiomyopathy. He remained asymptomatic throughout four years of follow-up. Through shared decision making, he continued to play competitively and is now a professional athlete. (Level of Difficulty: Advanced.).

An Unusual Case of Left Ventricular Outflow Tract Pseudoaneurysm.

Left ventricular outflow tract (LVOT) pseudoaneurysm is a rare condition with a wide range of causes and various clinical outcomes. The causes range from infections, trauma to the chest wall, and iatrogenic origins. We present a unique case of idiopathic LVOT pseudoaneurysm in a patient with no obvious clinical risk factors. (Level of Difficulty: Advanced.).

An Unusual Cause of Stroke: A "Forgotten" Intracardiac Catheter Discovered After 48 Years.

A 48-year-old man with recurrent cerebral ischemic events underwent transesophageal echocardiography to investigate cardioembolic sources because transthoracic echocardiography had spotted a dubious filamentous mass on the mitral valve. A venous umbilical catheter, lost during the hospital stay after his birth, was surprisingly identified in his cardiac chambers. (Level of Difficulty: Beginner.).

Right-to-left shunt via iatrogenic atrial septal defect requiring emergency surgical repair following cryoballoon atrial fibrillation ablation in a patient with arrhythmogenic right ventricular cardiomyopathy.

An 81-year-old woman with arrhythmogenic right ventricular cardiomyopathy underwent catheter ablation for atrial fibrillation and atrial flutter. Hypoxemia refractory to the administration of oxygen was seen after transseptal puncture. Transthoracic echocardiography revealed right to left shunt via an iatrogenic atrial septal defect (IASD) that was increased by tricuspid regurgitation flow. Her hypoxemia improved after IASD occlusion with the inflation of a venogram balloon catheter. Emergent surgical IASD closure was successfully performed. IASD after transseptal puncture for atrial fibrillation ablation infrequently causes severe complications that require emergent repair. Learning objective: Some cases requiring iatrogenic atrial septal defect (IASD) closure after atrial fibrillation (AF) ablation have been reported. We describe the case of an arrhythmogenic right ventricular cardiomyopathy patient with right to left shunt via an IASD which required emergent surgical repair after AF ablation. Right to left shunt after trans-septal puncture is rare, however it can be an emergent life-threatening complication. IASD occlusion with venogram balloon catheter is helpful for the diagnosis and the short-term solution.

Time-Velocity Integral of Left Ventricular Outflow Tract Predicts Worse Long-Term Survival in Pulmonary Arterial Hypertension.

Background: The time-velocity integral of the left ventricular outflow tract (TVILVOT) has been demonstrated to correlate with heart failure hospitalization and mortality, but the association of TVILVOT with the severity and prognosis of pulmonary arterial hypertension (PAH) has not been evaluated. Objectives: The aim of this study was to investigate the predictive value of baseline TVILVOT in PAH. Methods: A total of 225 consecutive patients with a diagnosis of incident PAH were prospectively studied and echocardiology-derived TVILVOT was measured at enrollment followed by right heart catheterization examination within 48 hours. Cox proportional hazards analysis was performed to assess the association between baseline variables and mortality. Results: During a median follow-up period of 33.8 months, 44 patients died of cardiovascular events. Baseline TVILVOT was significantly lower in the nonsurvivors compared with the survivors (P < 0.001). Baseline TVILVOT was positively correlated with stroke volume obtained by right heart catheterization (r = 0.709; P < 0.001), and inversely correlated with N-terminal pro-B-type natriuretic peptide (r = -0.533; P < 0.001), pulmonary vascular resistance (r = -0.423; P < 0.001). Multivariate analysis showed that baseline TVILVOT (hazard ratio: 0.856; 95% CI: 0.780-0.941; P = 0.001) was an independent predictor of cardiovascular mortality in PAH. Patients with a baseline TVILVOT <17.1 cm (median value) had a significantly worse survival than those with a baseline TVILVOT ≥17.1 cm (P < 0.001). Conclusions: The findings of this study suggest that noninvasive TVILVOT provides a practical method to assess the severity and predict long-term outcome of PAH.

Artificial Intelligence-Enabled Electrocardiogram Improves the Diagnosis and Prediction of Mortality in Patients With Pulmonary Hypertension.

Background: Pulmonary hypertension is a disabling and life-threatening cardiovascular disease. Early detection of elevated pulmonary artery pressure (ePAP) is needed for prompt diagnosis and treatment to avoid detrimental consequences of pulmonary hypertension. Objectives: This study sought to develop an artificial intelligence (AI)-enabled electrocardiogram (ECG) model to identify patients with ePAP and related prognostic implications. Methods: From a hospital-based ECG database, the authors extracted the first pairs of ECG and transthoracic echocardiography taken within 2 weeks of each other from 41,097 patients to develop an AI model for detecting ePAP (PAP > 50 mm Hg by transthoracic echocardiography). The model was evaluated on independent data sets, including an external cohort of patients from Japan. Results: Tests of 10-fold cross-validation neural-network deep learning showed that the area under the receiver-operating characteristic curve of the AI model was 0.88 (sensitivity 81.0%; specificity 79.6%) for detecting ePAP. The diagnostic performance was consistent across age, sex, and various comorbidities (diagnostic odds ratio >8 for most factors examined). At 6-year follow-up, the patients predicted by the AI model to have ePAP were independently associated with higher cardiovascular mortality (HR: 3.69). Similar diagnostic performance and prediction for cardiovascular mortality could be replicated in the external cohort. Conclusions: The ECG-based AI model identified patients with ePAP and predicted their future risk for cardiovascular mortality. This model could serve as a useful clinical test to identify patients with pulmonary hypertension so that treatment can be initiated early to improve their survival prognosis.

Late Transcatheter Aortic Valve Thrombosis Leading to Cardiogenic Shock.

A 67-year-old woman with prior transcatheter aortic valve replacement presented with worsening dyspnea. Imaging revealed transcatheter aortic valve thrombosis and aortic stenosis. Despite oral anticoagulation, she progressively deteriorated and developed cardiogenic shock. We highlight the Heart Team's role in treating this unusual late thrombosis. (Level of Difficulty: Intermediate.).

Curative Pericardiectomy in Interpheron-Resistant Severe Pericardial Erdheim-Chester Disease.

Erdheim-Chester disease is a rare clonal non-Langerhans cell histiocytosis with multisystemic involvement. It affects bones, large vessels, and retroperitoneum. Cardiac involvement is one of the main mortality predictors.1 We present an unusual case that debuted with cardiac tamponade and pericardial constriction requiring pericardiectomy for definitive control. (Level of Difficulty: Intermediate.).

Report of Accidental Anchoring of an Impella Device to a Perceval Bioprosthesis in a Patient.

We herein report a case in which we encountered complications when placing an Impella CP ventricular assist device (catheter-based ventricular assist device) in a patient with a Perceval bioprosthetic valve (sutureless valve). Specifically, the catheter-based ventricular assist device became anchored to the sutureless valve and needed to be removed under cardiopulmonary bypass. (Level of Difficulty: Advanced.).

An Integrative, Multiparametric Approach to Mitral Regurgitation Evaluation: A Case-Based Illustration.

Echocardiography is the first-line modality for assessing mitral regurgitation (MR). In addition to evaluation of the MR jet characteristics, echocardiography can provide quantitative parameters of MR severity. This case series illustrates the importance of integrating multiple parameters in the evaluation of MR and the role of multimodality imaging. (Level of Difficulty: Advanced.).

Intramyocardial Dissecting Hematoma: A Mechanical Complication Needing Surgical Therapy?

Intramyocardial dissecting hematoma is a form of cardiac rupture caused by myocardial infarction, percutaneous coronary intervention, or trauma. It is a cavity between myocardial fibers caused by partial rupture of the ventricular wall. Therapeutic management, including the timing for surgical approach, has not been standardized. We present a case series describing 4 patients. (Level of Difficulty: Intermediate.).

Acute Post-Traumatic Aortic Regurgitation.

A 16-year-old girl with a history of blunt chest trauma was admitted because of heart failure symptoms. Transthoracic echocardiography showed severe eccentric aortic regurgitation. Cardiac computed tomography revealed a pseudoaneurysm of the right sinus of Valsalva. We present a rare clinical presentation of a life-threatening condition. (Level of Difficulty: Intermediate.).

"Collar-like" neocommissure in mitral valve repair for extensive commissural prolapse.

Objectives: Mitral valve repair for extensive commissural prolapse is complex; several methods have been reported with variable results. To restore the leaflet area, we introduced an innovative and effective method rather than a simple folding technique through reconstructing a neocommissure with a "collar-like" plasty technique. Methods: From 2016 to 2021, 62 patients with mitral valve extensive commissural prolapse that received the "collar-like" plasty technique were included. Results: The mean age was 51.7 ± 13.8 years, and 38 (61%) were female. Postplasty transesophageal echocardiography showed the mean transvalvular pressure gradients were 3.8 ± 1.2 mm Hg, end diastolic peak flow velocity was 1.1 ± 0.3 m/s, and coaptation height was 7.9 ± 1.1 mm. During the follow-up period of 3 to 6 months, no deaths or cardiovascular-/valvular plasty-related adverse events occurred. All patients regained their heart function and returned to normal physical activities. Conclusions: The "collar-like" plasty technique can be safely performed for patients with extensive commissural prolapse. Echocardiography revealed that the technique can restore the leaflet area of low transvalvular pressure and flow velocity.