Surgical aortic valve replacement (SAVR) remains the standard of care for patients with chronic severe aortic regurgitation (AR). The introduction of a dedicated transcatheter heart valve for AR has fueled interest in using transcatheter aortic valve replacement to treat patients with isolated AR. We aimed to characterize the profile and outcomes of patients with symptomatic severe AR who underwent isolated SAVR. We conducted a retrospective, observational study of patients who underwent isolated SAVR for symptomatic severe AR at our institution. The primary outcome was in-hospital all-cause mortality. Patients were followed up with 30-day clinical and echocardiographic assessment. A total of 979 patients who underwent SAVR for severe AR between January 2015 and June 2021 were screened for eligibility, of whom 112 patients (11.4%) underwent isolated SAVR for symptomatic severe AR and were included in this analysis. Approximately 25% of patients were deemed to be at intermediate or high risk (n = 26 of 112). The primary outcome occurred in 2.7% of patients (n = 3 of 112). In-hospital stroke occurred in 2.7% of patients (n = 3 of 112), and new-onset atrial fibrillation occurred in 32.1% (n = 36 of 112). At 30-day follow-up, all-cause mortality occurred in 3.6% of patients (n = 4 of 112), and 0.8% (1 of 112) had >mild AR. In conclusion, in a tertiary referral center, the number of patients who underwent isolated SAVR for pure AR represented a small fraction of the overall SAVR patients. The vast majority were low risk and younger when compared with patients with severe aortic stenosis. SAVR yielded excellent short-term mortality and echocardiographic improvements.

Embolic myocardial infarction is an uncommon but increasingly recognized complication of infective endocarditis (IE). Although the incidence is low and ranges from 1% to 10%, the mortality rate is high (64%). The characteristics of septic embolism on presentation are nonspecific and usually are unrecognized by clinicians. This case report aims to build a high index of suspicion among clinicians for IE presenting with the complication of embolic myocardial infarction especially in patients with indwelling venous catheters.

A 47-year-old man presented with hematuria. Computed tomography( CT) showed a posterior mediastinal cyst. Chest magnetic resonance imaging showed a well defined mass with high intensity on T2-weighted images. Echocardiogram revealed severe aortic regurgitation, moderate mitral regurgitation and no continuity between the cyst and the pericardium. We performed aortic valve replacement, mitral annuloplasty and cyst resection after confirming it was not malignant by intraoperative rapid pathological examination. We performed sufficient and safe cyst resection through full sternotomy under cardiac arrest. Pathological examination revealed that cyst was bronchogenic. Bronchogenic cyst has malignant potential and it is very difficult to resect after presenting symptoms. We need to consider the differential diagnosis, the timing of operation and operative strategy.

The clinical impact of prosthesis-patient mismatch (PPM) in patients with small aortic annuli who underwent transcatheter aortic valve (AV) implantation with either balloon-expandable (BE) or self-expanding (SE) valves remains controversial. We assessed in-hospital and intermediate clinical outcomes in 573 patients with transfemoral transcatheter AV implantation with a small AV annulus, defined as an AV annulus area ≤430 mm2. A total of 337 patients treated with a 23-mm BE valve (SAPIEN 3, Ultra) were compared with 236 patients treated with a 26-mm SE valve (Evolut series). Valve-in-valve cases were excluded, and late echo follow-up (mean 674 ± 438 days) was assessed in a subset of 292 patients (51.0%). Well-matched BE and SE cohorts did not differ with respect to major in-hospital outcomes, other than a borderline increase in vascular complications and composite bleeding in patients with SE. Patients with BE had a higher incidence of severe PPM on discharge echocardiography (16.9% vs 6.8%, p <0.002). The mean AV gradient at 30 days was higher for patients with BE (12.2 ± 4.2 vs 6.2 ± 7.9 mm Hg, p <0.001) and at late follow-up (14.0 ± 8.2 vs 7.2 ± 3.5 mm Hg, p <0.001). The follow-up left ventricular ejection fraction and incidence of >mild aortic insufficiency were similar. All-cause mortality for the 2 cohorts was similar, with an overall mean (95% confidence interval) survival time of 61.2 months (57.8 to 64.5; p = 0.98). There were no significant survival differences between combined patients with BE and SE with no, moderate, or severe PPM, with an overall mean (95% confidence interval) survival time of 32.5 (30.5 to 34.5) months combining valve types (p = 0.23). In conclusion, despite an increased incidence of PPM with higher mean AV gradients that persist on late echocardiography in the BE cohort, patients with BE and SE with small aortic annuli have similar clinical outcomes at intermediate follow-up. Moderate and severe PPM had no impact on survival at a mean follow-up of 32.5 months.

Cardiac auscultation is a noninvasive, convenient, and low-cost diagnostic method for heart valvular disease, and it can diagnose the abnormality of the heart valve at an early stage. However, the accuracy of auscultation relies on the professionalism of cardiologists. Doctors in remote areas may lack the experience to diagnose correctly. Therefore, it is necessary to design a system to assist with the diagnosis. This study proposed a computer-aided heart valve disease diagnosis system, including a heart sound acquisition module, a trained model for diagnosis, and software, which can diagnose four kinds of heart valve diseases. In this study, a training dataset containing five categories of heart sounds was collected, including normal, mitral stenosis, mitral regurgitation, and aortic stenosis heart sound. A convolutional neural network GoogLeNet and weighted KNN are used to train the models separately. For the model trained by the convolutional neural network, time series heart sound signals are converted into time-frequency scalograms based on continuous wavelet transform to adapt to the architecture of GoogLeNet. For the model trained by weighted KNN, features from the time domain and time-frequency domain are extracted manually. Then feature selection based on the chi-square test is performed to get a better group of features. Moreover, we designed software that lets doctors upload heart sounds, visualize the heart sound waveform, and use the model to get the diagnosis. Model assessments using accuracy, sensitivity, specificity, and F1 score indicators are done on two trained models. The results showed that the model trained by modified GoogLeNet outperformed others, with an overall accuracy of 97.5%. The average accuracy, sensitivity, specificity, and F1 score for diagnosing four kinds of heart valve diseases are 98.75%, 96.88%, 99.22%, and 97.99%, respectively. The computer-aided diagnosis system, with a heart sound acquisition module, a diagnostic model, and software, can visualize the heart sound waveform and show the reference diagnostic results. This can assist in the diagnosis of heart valve diseases, especially in remote areas, which lack skilled doctors.

Transcatheter Aortic Valve Implantation in Multivalvular Heart Disease Abstract. The prevalence of multivaluvular heart disease is high in patients undergoing transcatheter aortic valve implantation (TAVI). The most common combination is aortic valve stenosis (AS) and mitral regurgitation, followed by the combination of AS with a tricuspid regurgitation or mitral stenosis. Grading of multivalvular disease is challenging and can quickly lead to underestimation of the disease stage. Therefore, a profound knowledge of pathophysiologic interactions is essential, and the patient should always undergo multimodal evaluation. After a successful TAVI intervention, secondary heart valve defects may improve, deteriorate, or remain unchanged. Due to the still sparse scientific data in this field, the role of the heart team remains central to provide the patient with an individually adapted therapy plan.

Left ventricular outflow stenosis can develop at the supravalvular, valvular, and subvalvular levels. Resection of strictures at the diffuse subvalvular level is very difficult. In such pathologies, Konno-Rastan procedure provides very successful solutions as an anterior aortoventriculoplasty method. In this article, we performed anterior aortaventriculoplasty surgical treatment for tunnel type left ventricular outflow tract stenosis, recurrent subvalvular discrete membrane, and aortic regurgitation in an adult patient with a history of partial atrioventricular septal defect repair and subvalvular discrete membrane resection operation in early childhood. The Konno-Rastan procedure, which we applied to the redo case, which is rarely used in adult patients and rarely seen in the literature, is shared.

Background: Mucopolysaccharidosis Type II (MPS II) is a rare, progressive and ultimately fatal X-linked lysosomal storage disorder caused by mutations in the iduronate-2-sulfatase (IDS) gene. This report conducted a retrospective analysis to investigate the clinical characteristics, genotypes and management strategies in a large cohort of Chinese patients with MPS II. Methods: In this study, we explored 130 Chinese patients with MPS II between September 2008 and April 2022. Clinical manifestations, auxiliary examination, IDS pathogenic gene variants and IDS enzyme activity, surgical history were analysed in the study. Results: A total of 130 patients were enrolled and the mean age at diagnosis was 5 years old. This study found the most common symptoms in our patients were claw-like hands, followed by coarse facial features, birthmarks (Mongolian spot), delayed development, inguinal or umbilical hernia. The most commonly cardiac manifestations were valve abnormalities, which were mitral/tricuspid valve regurgitation (71.9%) and aortic/pulmonary valve regurgitation (36.8%). We had found 43 different IDS pathogenic gene variants in 55 patients, included 16 novel variants. The variants were concentrated in exon 9 (20% = 11/55), exon 3 (20% = 11/55) and exon 8 (15% = 8/55). A total of 50 patients (38.5%) underwent surgical treatment, receiving a total of 63 surgeries. The average age of first surgery was 2.6 years, and the majority of surgery (85.7%, 54/63) was operated before 4 years old. The most common and earliest surgery was hernia repair. Three patients were died of respiratory failure. Conclusion: This study provided additional information on the clinical, cardiac ultrasound and surgical procedure in MPS II patients. Our study expanded the genotype spectrum of MPS II. Based on these data, characterization of MPS II patients group could be used to early diagnosis and treatment of the disease.

There is no consensus regarding mitral valve management during surgical ventricular restoration (SVR) for ischemic cardiomyopathy. We compared the impact of SVR with mitral valve repair (MVr) vs replacement (MVR) on postoperative outcomes and long-term survival in ischemic cardiomyopathy and mitral regurgitation patients. This study included 112 patients who underwent SVR from 2009 to 2018 with MVr (n = 75) or MVR (n = 37). Patients who had MVR had higher Euro SCORE II, dyspnea class, a lower ejection fraction, higher pulmonary artery systolic pressure, higher grade of preoperative mitral and tricuspid regurgitation, and higher end-diastolic and end-systolic diameters. Intra-aortic balloon pump was more commonly used in patients with MVR. Hospital mortality occurred in 7 (9.33%) patients in the MVr group vs 3 (8.11%) in the MVR group (P > .99). Freedom from rehospitalization at 1, 5, and 7 years was 87%, 76%, and 70% in the MVr group and 83%, 61%, and 52% in the MVR group (P = .191). Survival at 1, 5, and 7 years was 88%, 78%, and 74% in the MVr group and 88%, 56%, and 56% in the MVR group (P = .027). Adjusted survival did not differ between groups.MVr or MVR are valid options in patients undergoing SVR, with good long-term outcomes.

There have been several advances in the diagnosis and management of valvular heart disease (VHD) over the last decade. These have been reflected in the latest European and North American guidelines, although both contain significant similarities and differences. In this review, we highlight the important overlaps and variations between the updated guidelines and their previous versions to help guide the general cardiologist.

Although veno-arterial extracorporeal membrane oxygenation (VA-ECMO) has been used to aid myocardial recovery in patients with postcardiotomy cardiogenic shock (PCCS), it has been associated with adverse effects. The combined use of VA-ECMO and Impella (ECPELLA) for PCCS, however, has been reported to be efficacious with few reports of thromboembolic events. We present a case of aortic thrombosis with visceral malperfusion during ECPELLA management for PCCS. We performed the Bentall procedure, mitral valve repair, tricuspid annuloplasty, and coronary artery bypass graft on a 73-year-old man admitted with congestive heart failure caused by annuloaortic ectasia, along with severe aortic and mitral regurgitation. VA-ECMO and Impella were required, since the cardiopulmonary bypass weaning was difficult. Impella was removed on postoperative day 4. On postoperative days 5 and 6, laboratory data showed worsening renal dysfunction, lactate levels, and acidosis. Contrast-enhanced computed tomography showed thrombosis in the celiac and superior mesenteric arteries. Aortic thrombectomy was performed. Hyperkalemia, caused by a reperfusion injury, resulted in ventricular fibrillation. Continuous hemodiafiltration improved the hyperkalemia. However, irreversible acidosis progressed, and the VA-ECMO flow rate could not be sustained. On postoperative day 7, the patient died. Perioperative use of Impella for PCCS may be effective in improving postoperative cardiac function. When sudden organ failure is observed after surgery, it is necessary to not only keep the exacerbation of cardiogenic shock in mind, but also the possibility of thrombosis.

Group A Streptococcus is a common cause of pharyngitis, scarlet fever, glomerulonephritis, and various skin infections. However, there have only been a few reports of Group A Streptococcus (GAS) associated with infective endocarditis. We present a case of a 31-year-old male who presented to the hospital with a few days of weakness and myalgias after recently receiving treatment with azithromycin for GAS pharyngitis as an outpatient. The patient was admitted to the intensive care unit in septic shock from Group A beta-hemolytic Streptococcus bacteremia and acute hypoxic respiratory failure. He was found to have new severe aortic insufficiency from infective endocarditis. He underwent urgent partial aortic valve annuloplasty reconstruction and incision and drainage of root abscess with intraoperative cultures growing gram positive cocci consistent with GAS. The majority of cases of infective endocarditis are caused by Staphylococcus aureus, virdians group Streptococci and Streptococcus gallolyticus, while Streptococcus pneumoniae and Streptococcus pyogenes have the lowest prevalence of infective endocarditis. Penicillin remains the first line treatment for GAS pharyngitis. However, in cases of severe penicillin allergy, macrolides should be avoided given the increasing rate of macrolide resistance GAS strains. This case emphasizes the importance of using beta-lactams as first line therapy for GAS pharyngitis to avoid harmful complications and infection recurrence.

The pharmacotherapy of heart failure has evolved in recent years and with the aid of new classes of drugs symptomatic and prognostic improvements can be achieved in patients with heart failure. Heart failure is particularly frequently associated with coronary artery disease or higher grade, often functional valve defects. In the context of the underlying disease, the operative risk is often increased, so that interventional treatment is preferred over surgical treatment options in interdisciplinary heart teams. Promising approaches with very different challenges are emerging for interventional myocardial revascularization and percutaneous correction of high-grade aortic valve stenosis or functional mitral or tricuspid valve regurgitation. It has consistently been shown that an elaborate diagnostic work-up and differentiated patient selection are decisive to achieve a prognostic or symptomatic benefit in these patients using interventional treatment. While awaiting further study data on this topic, the integration of a multidisciplinary heart team is essential to ensure a complementary and balanced therapeutic approach for patient-centered care in this complex patient population.

Bartonella sp. are the most common causes of culture-negative infective endocarditis (IE) cases in the United States. Although, infection-related glomerulonephritis can frequently mimic primary vasculitis due to pauci-immune pattern, majority of previously reported cases of Bartonella henselae-associated glomerulonephritis have immune-complex deposits on immunofluorescence. We present a rare case of B henselae IE-related pauci-immune necrotizing glomerulonephritis. Timely recognition of this atypical presentation led to appropriately directed medical therapy.

Patients suffering from end-stage heart failure tend to have high mortality rates. With growing numbers of patients progressing into severe heart failure, the shortage of available donors is a growing concern, with less than 10% of patients undergoing cardiac transplantation (CTx). Fortunately, the use of left ventricular assist devices (LVADs), a variant of mechanical circulatory support has been on the rise in recent years. The expansion of LVADs has led them to be incorporated into a variety of clinical settings, based on the goals of therapy for patients ailing from heart failure. However, with an increase in the use of LVADs, there are a host of complications that arise with it. One such complication is the development and progression of aortic regurgitation (AR) which is noted to adversely influence patient outcomes and compromise pump benefits leading to increased morbidity and mortality. The underlying mechanisms are likely multifactorial and involve the aortic root-aortic valve (AV) complex, as well as the LVAD device, patient, and other factors, all of them alter the physiological mechanics of the heart resulting in AV dysfunction. Thus, it is imperative to screen patients before LVAD implantation for AR, as moderate or greater AR requires a concurrent intervention at the time of LVADs implantation. No current strict guidelines were identified in the literature search on how to actively manage and limit the development and/or progression of AR, due to the limited information. However, some recommendations include medical management by targeting fluid overload and arterial blood pressure, along with adjusting the settings of the LVADs device itself. Surgical interventions are to be considered depending on patient factors, goals of care, and the underlying pathology. These interventions include the closure of the AV, replacement of the valve, and percutaneous approach via percutaneous occluding device or transcatheter aortic valve implantation. In the present review, we describe the interaction between AV and LVAD placement, in terms of patient management and prognosis. Also it is provided a comprehensive echocardiographic strategy for the precise assessment of AV regurgitation severity.

Transcatheter closure of Perimembranous VSDs (PMVSD) remains challenging particularly in infants. The aim of this study is to evaluate the efficacy and safety of transfemoral PMVSD device closure in infants weighing ≤ 10 kg in a single centre. Retrospective review of departmental databases and medical charts to define patient cohort and collect demographic, procedural and follow-up data. Between July 2014 and March 2021, 16 patients underwent attempted transfemoral PMVSD device closure (12 retrograde) at a median age of 11 months (interquartile range [IQR] 9-15.5) and a median weight of 8.3 kg (IQR 7.2-9.5). All patients were either symptomatic, had progressive left heart dilation or had VSD associated valve regurgitation. Median defect size on pre-procedural transoesophageal echocardiography was 6.8 mm (IQR 6-8.5). Median device waist size was 6 mm (IQR 4.5-8). Successful device placement was achieved in 14 patients (88%). One patient developed moderate aortic and tricuspid valve regurgitation upon retrograde and antegrade device deployment, respectively, and subsequently underwent surgical closure. The second patient developed progressive aortic regurgitation (AR) 2 days post procedure, and also underwent surgical removal with no residual AR. There was no cases of device embolization and no femoral arterial compromise. On median follow-up of 40.5 months (IQR 25-64), none of the patients developed complete heart block. Three patients (18.75%) had small residual shunts at latest follow-up which have not required any further intervention. Device closure of PMVSD's in children weighing ≤ 10 kg is feasible and safe with good procedural success rates. Use of both the antegrade and retrograde approaches may be necessary depending on anatomical variances.

Left atrial wall dissection is a rare entity with controversial management approaches. We report the case of an 85-year-old woman with a mitral bioprosthetic admitted for heart failure. Diagnosis of atrial septum dissection, severe aortic stenosis, and paravalvular mitral regurgitation was established. A percutaneous approach provided clinical improvements.

A patient with a history of Crohn's disease on infliximab presented to the hospital with sepsis and a new heart murmur. He was found to have native aortic valve infective endocarditis from a rare species of group D Streptococcusin his blood. The patient was also noted to be in an acute flare of Crohn's disease. The hospital course was complicated by florid heart failure from acute aortic insufficiency. He eventually improved after source control and appropriate antibiotic therapy. S. pasteuranis bacteremia and endocarditis are attributable to the patient's immunocompromised state as a result of infliximab treatment. While S. pasteuranis is infrequently grown in blood cultures, it is commonly found in normal gut flora. We hypothesize that it gained access to the bloodstream through the epithelium in the terminal ileum, which was inflamed due to an acute flare of Crohn's disease.