Objective Affecting 1 in 500 individuals; Hypertrophic cardiomyopathy (HCM) is an autosomal dominant cardiovascular disorder which is prevalent throughout the world. Surgical myectomy and alcohol septal ablation (ASA) are two methods currently used for the management of drug refractory Hypertrophic obstructive cardiomyopathy (HOCM). ASA may prove to be a useful, less invasive tool when confronting patients with HOCM especially those who are more elderly or deemed to be a higher surgical risk. Methods Electronic literature search was conducted to identify relevant articles that discussed invasive methods to treat drug refractory HOCM. No limits were placed on timing of the publication or the type of article. Key words and MeSH terms were used to conduct the search and the results were summarized in the relevant section. Results Current evidence suggests that alcohol septal ablation is a safe and effective procedure in treating patients with HOCM with similar short- and long-term outcomes when compared with surgical myectomy. Selection of patient with appropriate assessment is the key for satisfactory outcomes. Conclusion ASA has been shown to be a safe and reliable procedure; advanced imaging techniques and dedicated multi-disciplinary teams can be used to carefully select patients with HOCM. Though surgical myectomy is recommended as gold standard treatment for drug refractory HOCM, however, ASA may play an increasing role in the near future due an ageing population; both ASA and SM can have a synergistic effect in treating those who are affected by HOCM.
Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly. The aorta and the main pulmonary arterial trunk arises predominantly from the left ventricle(LV) and is associated with a malaligned ventricular septal defect(VSD), various degrees of hypoplasia of the right ventricle, and presence or absence of pulmonary stenosis. Bi-ventricular repair is the preferred treatment option whenever possible. Various techniques for bi-ventricular repair have been described. The best option for DOLV correction is by translocating the pulmonary root to the right ventricle(RV). In this series, we report four patients who underwent biventricular repair of DOLV in our institute with excellent outcomes. All patient details were collected from the institute patient record system. Echocardiographic data were obtained from the records. Intraoperative charts were reviewed for further information on the surgical procedure and cardiopulmonary bypass. Postoperative data included survival, functional status and followup echocardiography. Of the four children, three underwent pulmonary root translocation and one child underwent Reparation al etage Ventriculaire(REV) procedure. There was no mortality and all children are in stable clinical condition in the recent follow-up and no re-operations or interventions were required following primary surgical correction. Thus DOLV is anatomically and surgically a challenging subset. Pulmonary root translocation in this anatomy is technically challenging but safe and superior option when compared to other alternative surgical procedures and it can be performed with excellent results, even in infants.
While there is significant awareness regarding droplet and contact transmission, aerosols are generally underestimated as a potential mode of transmission of SARS-Cov-2 infection. With the gradual resumption of cardiac surgical activities, the cardiac surgical operating room will become an important potential source of infection to the cardiac surgeon and other healthcare workers participating in the operation. There is also diminished awareness about the different aerosol generating procedures (AGP) in the cardiac surgical operating room. In this mini-review we intend to highlight the various aerosol generating procedures that are common in cardiac surgery. This will help increase the awareness among surgeons to AGP. A practical approach to taking preventive measures have also been discussed.
Background: With the limited number of available suitable donor hearts resulting in plateaued numbers of heart transplantations, short- and long-term mechanical circulatory support devices, including the implantation of total artificial hearts (TAH) are modalities that are increasingly being used as treatment options for patients with end-stage heart failure. The superior vena cava syndrome has been described in this context in various disease processes. We report successful venoplasty for superior vena cava syndrome in a patient with a TAH. Case Presentation: A 65 years old man with a history of non-ischemic cardiomyopathy had received a left ventricular assist device, and then two years later underwent orthotopic heart transplantation using the bicaval anastomosis technique. The post procedural course was complicated by primary graft failure, resulting in the need for the implantation of a TAH. About 5 months after the TAH implantation he started to develop complications such as volume retention, swelling of the upper extremities, and was diagnosed to have a superior vena cava syndrome. The patient underwent a successful venoplasty of his superior vena cava by interventional radiology with resolution of upper body edema, normalization of renal and liver function. Conclusion: Potential fatal complications caused by catheter or wire entrapment in the right sided mechanical valve of a TAH have been reported. We describe a safe method for the treatment of superior vena cava syndrome in patients with TAH.
Objectives The impact of the COVID 19 pandemic on the treatment of patient with aortic valve stenosis is unknown and there is uncertainty on the optimal strategies in managing these patients. Methods This study is supported and endorsed by the Asia Pacific Society of Interventional Cardiology. Due to the inability to have face to face discussions during the pandemic, an online survey was performed by inviting key opinion leaders ( cardiac surgeon/interventional cardiologist/echocardiologist) in the field of transcatheter aortic valve implantation (TAVI) in Asia to participate. The answers to a series of questions pertaining to the impact of COVID-19 on TAVI were collected and analyzed. These led subsequently to an expert consensus recommendations on the conduct of TAVI during the pandemic Results The COVID 19 pandemic had resulted in a 25% (10-80) reduction of case volume and 53% of operators required triaging to manage their patients with severe aortic stenosis. The two most important parameters used to triage were symptoms and valve area. Periprocedural changes included the introduction of teleconsultation, pre-procedure COVD 19 testing, optimization of pre-tests and catheterization laboratory set up. In addition, length of stay was reduced from a mean of 4.4 to 4 days. Conclusion The COVID-19 pandemic has impacted on the delivery of TAVI services to patients in Asia. This expert recommendations on best practices may be a useful to guide to help TAVI teams during this period until a COVID 19 vaccine becomes widely available
We report the successful endovascular repair of a rare case of aortic rupture caused by axillary intra-aortic balloon pump insertion failure. A 38-year-old Jehovah’s Witness female with situs inversus totalis was referred to our hospital for acute decompensated heart failure. We placed an axillary intra-aortic balloon pump for circulatory support. However, an exchange was required due to balloon malfunction (kinked driveline). Unfortunately, the exchange was complicated by an iatrogenic aortic rupture along with large hematoma compressing the trachea. Emergent endovascular repair was performed successfully without any blood transfusion. Postoperative computed tomography showed a successfully repaired aorta and resolving hematoma.
Diastolic dysfunction (DD) is reported to affect up to 35% of the adult general population. The consequence of progressive DD is heart failure with preserved ejection fraction (HFpEF). Coronary microvascular dysfunction (CMD) has been suggested as one of the pathologic mechanisms leading to HFpEF. We investigated whether there was an association between coronary microvascular function and echocardiographic indices of left ventricular diastolic function in patients with chest pain and unobstructed coronary arteries (CPUCA). This retrospective observational study recruited patients referred to cardiology clinics assessment of chest pain who subsequently underwent assessment via CT coronary angiogram (CTA). Coronary microvascular dysfunction was determined by myocardial blood flow reserve (MBFR; <2.0) using myocardial contrast echocardiography. Echocardiographic indices of diastolic function (septal mitral annular e’; septal mitral annular E/e’) were measured from baseline transthoracic echocardiogram. 149 patients (52% men) with a mean age 59.7(9.5) years were recruited. Mean (standard deviation) MBFR was 2.2 (0.51). 37% (55/149) had MBFR<2.0. Median [interquartile range] septal mitral annular e’ velocity and septal mitral annular E/e’ were 7.6 cm/s [6.2, 8.9] and 9.5 [7.5, 10.8] respectively. Univariate regression analysis showed only age was a significant predictor of increasing septal mitral annular E/e’ (=+0.20 95% CI 0.13, +0.28, p<0.001) but not MBFR. Multivariable analysis also showed no association between these septal mitral annular E/e’ and MBFR after adjustment for cardiovascular risk factors. There was no relationship found between echocardiographic indices of left ventricular diastolic function and coronary microvascular function.
Severe recurrent mitral regurgitation (MR) within 1 year of mitral valve repair is usually attributed to a technical issue with the original repair procedure. However, when artificial chordae are employed to correct mitral valve prolapse, ventricular remodeling (i.e. decreased ventricular size) can lead to recurrent prolapse and valve dysfunction. To highlight this phenomena, we present 2 patients who experienced early failure after undergoing mitral valve repair with artificial chordae.
After its first description in Wuhan (China), SARS-CoV-2 the agent of coronavirus disease 2019 (COVID-19) rapidly spread worldwide. Previous studies suggested that pets could be susceptible to SARS-CoV-2. Here, we investigated the putative infection of SARS-CoV-2 in 22 cats and 11 dogs from owners previously infected or suspected of being infected by SARS-CoV-2. For each animal, rectal, nasopharyngeal swabs and serum were taken. Swabs were submitted to RT-qPCR assays targeting 2 genes of SARS-CoV-2. All dogs were tested SARS-CoV-2 negative. One cat was tested positive by RT-qPCR on rectal swab. Nasopharyngeal swabs from this animal were tested negative. This cat showed mild respiratory and digestive signs. Serological analysis confirm the presence of antibodies against the SARS-CoV-2 in the two serum samples taken 10 days apart. Genome sequence analysis revealed that the cat SARS-CoV-2 belongs to the phylogenetic clade A2a like most of the French human SARS-CoV-2. This study reports for the first time the natural infection of a cat in France (near Paris) probably through their owners. There is currently no evidence that cats can spread COVID-19 and owners should not abandon their pets or compromise their welfare.
Less invasive techniques for cardiothoracic surgical procedures are designed to limit surgical trauma, but technical requirements and preoperative planning are more demanding than those for conventional sternotomy. Patient selection, interdisciplinary collaboration, and surgical skills are key factors for procedural success. Aortic valve replacement is frequently performed through an upper hemisternotomy, but the right anterior minithoracotomy represents an even less traumatic, technical advancement. Preoperative assessment of the ascending aorta in relation to the sternum is mandatory to select patients and the intercostal access site. This description of the surgical technique focuses on the specific procedural details including the obligatory planning with computed tomography, and our cannulation strategy. We also sought to define the anatomical ascending aorto-sternal relationship, as it is of utmost importance in preoperative computed tomographic planning.
4 cases have been reported from a tertiary referral hospital in Tehran, of which 3 have confirmed COVID-19 infection and one has been highly suspicious. The cases describe different cardiovascular presentations that can be linked to COVID-19 infection, including myocardial infarction, in-stent restenosis, myopericarditis and tamponade, and aortic dissection.
Predicting the energetics of chemical transformations requires localizing stationary points on a potential energy surface. Whereas educts and products of a chemical reaction may be known, transition state optimization is challenging, as good guesses may be unavailable. Extending stationary point searches to excited states leads to additional difficulties as several states may be close in energy, requiring efficient state-tracking. Herein we report the implementation of pysisyphus, an external optimizer, that allows not only the localization of stationary points in the ground state, but also for excited states by providing several state-tracking algorithms. Pysisyphus offers all necessary tools for calculating reaction paths starting from the optimization of the reactants, running chain-of-states methods like the nudged elastic band or the growing string method with subsequent transition state optimization and a concluding intrinsic reaction coordinate calculation.
Background: It has long been known that nasal inoculation with influenza A virus produces asymptomatic to febrile infections. Uncertainty persists about whether these infections are sufficiently similar to natural infections for studying human-to-human transmission. Methods: We compared influenza A viral aerosol shedding from volunteers nasally inoculated with A/Wisconsin/2005 (H3N2) and college community adults naturally infected with influenza A/H3N2 (2012-2013), selected for influenza-like illness with objectively measured fever or a positive Quidel QuickVue A&B test. Propensity scores were used to control for differences in symptom presentation observed between experimentally and naturally infected groups. Results: Eleven (28%) experimental and 71 (86%) natural cases shed into fine particle aerosols (p<0.001). The geometric mean (geometric standard deviation) for viral positive fine aerosol samples from experimental and natural cases was 5.1E+3 (4.72) and 3.9E+4 (15.12) RNA copies/half hour, respectively. The 95th percentile shedding rate was 2.4 log10 greater for naturally infected cases (1.4E+07 versus 7.4E+04). Certain influenza-like illness related symptoms were associated with viral aerosol shedding. The almost complete lack of symptom severity distributional overlap between groups did not support propensity score adjusted shedding comparisons. Conclusions: Due to selection bias, the natural and experimental infections had limited symptom severity distributional overlap precluding valid, propensity score adjusted comparison. Relative to the symptomatic naturally infected cases, where high aerosol shedders were found, experimental cases did not produce high aerosol shedders. Studying the frequency of aerosol shedding at the highest observed levels in natural infections without selection on symptoms or fever would support helpful comparisons.
The International Classification of Diseases (ICD) provides a common language for use worldwide as a diagnostic and classification tool for epidemiology, clinical purposes and health management. Since its first edition, the ICD has maintained a framework distributing conditions according to topography, with the result that some complex conditions, such as allergies and hypersensitivity disorders (A/H) including anaphylaxis, have been poorly represented. The change in hierarchy in ICD-11 permitted the construction of the pioneer section addressed to A/H, which may result in more accurate mortality and morbidity statistics, including more accurate accounting for mortality due to anaphylaxis, strengthen classification, terminology and definitions. The ICD-11 was presented and adopted by the 72nd World Health Assembly in May 2019 and the implementation is ongoing worldwide. We here present the outcomes from an online survey undertaken to reach out the allergy community worldwide in order to peer review the terminology, classification and definitions of A/H introduced into ICD-11 and to support their global implementation. Data are presented here for 406 respondents from 74 countries. All of the sub-sections of the new A/H section of the ICD-11 had been considered with good accuracy by the majority of respondents. We believe that, in addition to help during the implementation phase, all the comments provided will help to improve the A/H classification and to increase awareness by different disciplines of what actions are needed to ensure more accurate epidemiological data and better clinical management of A/H patients.
Are all Non-sustained Ventricular Tachycardia the Same in Hypertrophic Cardiomyopathy Risk Stratification for Sudden Cardiac Death?Mohamad Khaled Sabeh MD1, Marwan M. Refaat MD21Cardiac Arrhythmia Service, Massachusetts General Hospital, Boston, Massachusetts - USA2Division of Cardiology, Department of Internal Medicine, American University of Beirut Medical Center Beirut, LebanonRunning Title: NSVT in HCM SCD Risk StratificationWords (excluding references): 664Disclosures: NoneFunding: NoneKeywords: Hypertrophic Cardiomyopathy, Non-sustained Ventricular Tachycardia, Cardiac Arrhythmias, Cardiovascular DiseasesCorrespondence:Marwan M. Refaat, MD, FACC, FAHA, FHRS, FASE, FESC, FACP, FRCPAssociate Professor of MedicineDirector, Cardiovascular Fellowship ProgramDepartment of Internal Medicine, Cardiovascular Medicine/Cardiac ElectrophysiologyDepartment of Biochemistry and Molecular GeneticsAmerican University of Beirut Faculty of Medicine and Medical CenterPO Box 11-0236, Riad El-Solh 1107 2020- Beirut, LebanonFax: +961-1-370814Clinic: +961-1-350000/+961-1-374374 Extension 5800Office: +961-1-350000/+961-1-374374 Extension 5353 or Extension 5366 (Direct)Email: firstname.lastname@example.orgCardiomyopathies with reduced systolic function predispose to sudden cardiac death (SCD) and many studies helped in decreasing that risk by Implantable Cardioverter Defibrillator (ICD) implantation and pharmacologic management (1-4). Many types of cardiomyopathies with preserved systolic function, including hypertrophic cardiomyopathy (HCM), can predispose to malignant ventricular arrhythmias and SCD. HCM is the most common inherited cardiac disease that affects 1 in 200 live births (5,6). SCD remains one of the main causes of death in HCM and the SCD rate peaks in early adulthood (7-14). Data from ICDs suggest that SCD in HCM is most commonly caused by ventricular fibrillation (VF) (15). One major clinical challenge is identifying patients at risk for SCD. Multiple studies showed that non-sustained ventricular tachycardia (NSVT) is a risk actor for SCD (16,17). However the strength of the data was variable across these studies due to difference in populations and the low sensitivity of Holter ECG. Moreover, other studies looked at the rate and duration of the ventricular arrhythmias and their relationship to SCD in HCM (17-19) yet the effect of the morphology of NSVT on SCD has not been well investigated.In this single center study Adduci et al . explore the prognostic impact of different NSVT morphologies in a cohort of 109 consecutive HCM patients. The study included patients who had an ICD implanted in the authors’ institution from January 2001 to December 2018. The ICDs were mostly implanted for primary prevention in HCM patient with 1) one or more risk factor including maximal LV thickness ≥30 mm, family history of SD in at least 1 first-degree relative <50 years of age, non-sustained ventricular tachycardia (NSVT), recent (≤ 6 months) unexplained syncope, 2) hypotensive blood pressure during exercise with at least one additional major risk factor for SD 3) end-stage HCM regardless of other established risk markers of SCD. Devices were interrogated on evaluation every 3 to 6 months and the data was assessed for appropriate or inappropriate ICD therapies. Two independent electrophysiologists analyzed the ICD near field and far field EGMs from the ventricular tachycardia runs. They classified the VTs as either monomorphic (MMVT) or polymorphic (PMVT).During a mean follow up of 71+/- 48 months, 377 NSVT episodes of NSVT were retrieved from ICD memory in 46 patients; of these episodes, 7(2%) were polymorphic and 370 (98%) were monomorphic (MM). The mean HR of The MM NSVT had an average HR of 171+/- 32 BPM and lasted for 17 +/- 12 beats while the PMVT were faster at 241BPM +/- and longer at 28+/- 16 beats. The appropriate intervention rate was 5.1% per year and interestingly NSVT did not predict the occurrence of ICD therapy. However patients with polymorphic NSVT had a statistically higher risk for ICD intervention as compared to monomorphic NSVT. Further analysis noted a trend for increased risk of ICD therapy with patients with >1 NSVT morphology. Moreover 75% of the treated VTs had been previously observed as NSVT.Risk stratification is very important in this young patient population; decreasing the risk threshold for ICD implants leads to missed arrhythmias and bad outcomes while increasing it increases the risk for complications from unnecessarily implanted devices. There are several types of ICDs: Transvenous ICD, Subcutaneous ICD and Extravascular ICD. The results of this study suggest that the risk of SCD in patients with PMVT and/or NSVT with multiple morphologies is different from that of patients with a MMVT, and that the presence of short MMVT doe not predict the future ICD therapies. As such, one may consider a conservative approach in low-risk patients with short bursts of slow MM NSVT, and a more aggressive approach in patients with frequent, rapid rate burst of PMVT. Although this study suggests that different NSVT morphologies affect the prognosis in HCM patients, the low number of events lacked the statistical power to redefine ICD candidacy. Larger multicenter studies are needed to confirm these findings and to help delineate the “at risk patients” who would truly benefit from ICDs.
A Cardiac Sodium Channel Mutation Associated with Epinephrine-Induced Marked QT-ProlongationMohamad N. El Moheb MD1, Marwan M. Refaat MD21Division of Trauma Emergency Surgery and Surgical Critical Care, Massachusetts General Hospital, Boston, Massachusetts - USA2Division of Cardiology, Department of Internal Medicine, American University of Beirut Medical Center Beirut, LebanonRunning Title: SCN5A mutation associated with epinephrine-induced LQTSWords (excluding references): 746Disclosures: NoneFunding: NoneKeywords: Long QT Syndrome, Genetics, Variants, Cardiac Arrhythmias, Cardiovascular DiseasesCorrespondence:Marwan M. Refaat, MD, FACC, FAHA, FHRS, FASE, FESC, FACP, FRCPAssociate Professor of MedicineDirector, Cardiovascular Fellowship ProgramDepartment of Internal Medicine, Cardiovascular Medicine/Cardiac ElectrophysiologyDepartment of Biochemistry and Molecular GeneticsAmerican University of Beirut Faculty of Medicine and Medical CenterPO Box 11-0236, Riad El-Solh 1107 2020- Beirut, LebanonFax: +961-1-370814Clinic: +961-1-350000/+961-1-374374 Extension 5800Office: +961-1-350000/+961-1-374374 Extension 5353 or Extension 5366 (Direct)Email: email@example.comThe hereditary long QT syndrome (LQTS) is an important cause of polymorphous ventricular tachycardia (torsades de pointes) and sudden cardiac death in otherwise young and healthy individuals. Clinically, this condition is caused by delayed ventricular repolarization and manifests as an abnormally prolonged QT interval on the electrocardiogram (ECG). The most common subtypes of LQTS are LQT1, LQT2, and LQT3 (1-10). The life-threatening arrhythmias occur most frequently during exercise in LQT1, upon auditory stimulation or emotional stress in LQT2, and at rest or during sleep in LQT3 (11). Patients with LQT1 have a mutation in the KCNQ1 gene which codes for the subunit of the slow outward potassium current channel (IKs) while patients with LQT3 have a mutation in the SCN5A gene, which codes for the cardiac voltage-dependent sodium channel (INa) (12). LQT1-affected individuals are more vulnerable to β-adrenergic modulation than LQT3-affected individuals. Exercise and epinephrine-infusion ECG tests are therefore useful in differentiating between the LQTS subtypes and optimizing therapeutic strategies in order to prevent sudden cardiac death. While beta-blockers have been established as the standard of care for the treatment of the LQT1 and LQT2 subtypes, their use in LQT3 remains controversial (13, 14). A new missense mutation has been recently identified in the SCN5A-encoding INA channels and was found to be associated with sinus node dysfunction and epinephrine-induced QT prolongation (1). This atypical phenotype of LQT3 has so far been observed in only one patient. Whether other mutations exist that can cause a similar manifestation has yet to determined.In the current issue of the Journal of Cardiovascular Electrophysiology, Nakajima et al. describe a family with LQT3 that exhibited epinephrine-induced marked QT prolongation. The SCN5A V1667I mutation was found to be responsible for this atypical phenotype which resulted in prolongation of the QT interval in the proband as well as in family members carrying the mutation. The SCN5A V1667I mutation is a gain of function mutation located in domain IV-segment 5 (DIV-S5) of the sodium channel encoding SCN5A gene. To elucidate the pathophysiology of the disease, the authors transfected a human kidney cell line (tsA-201) to induce expression of wild-type and mutated sodium channels and measured the membrane sodium currents (INA). They showed that SCN5A V1667I mutation was associated with larger INA peak density, depolarizing shift in steady-state inactivation (SSI) leading to increased window current, and accelerated recovery from depolarization. Additionally, an increased hump in the INA of V1667I mutant cells (V1667I-INA) was observed during a ramp pulse protocol consistent with increased window current. There was no difference in fast inactivation rate and steady-state activation between the V1667I-INA and wild-type INA(WT-INA). The authors further examined the effects of protein kinase A (PKA) activation on V1667I-INA to mimic the effect of epinephrine. PKA activation resulted in a less significant hyperpolarizing shift in SSI in V1667I-INA compared to WT-INA leading to increased window current. Additionally, V1667I mutation was found to be associated with accelerated recovery from depolarization, and increased hump during ramp pulse protocol in the setting of PKA activation. Chen et al. have also reported the case of an individual with a mutation in SCN5A who exhibited marked QT-prolongation after epinephrine infusion (1). However, contrary to the SCN5A V1667I mutation described by Nakajima et al, the SCN5A V2016M defect was a loss of function mutation causing a decrease in INA peak density. The clinical manifestations of the SCN5A mutations described by Chen et al. and Nakajima et al. are more comparable to individuals with the LQT1 subtype than those with the LQT3 subtype. Therefore, it should be considered whether certain patients with SCN5A would benefit from beta-blocker therapy.Overall, the authors should be commended on their efforts to describe for the first time a family with the SCN5A V1667I mutation and show that this mutation is associated with epinephrine-induced marked QT prolongation. The authors have also provided important insight into the electrophysiological properties of the mutant channels and the structure-function relationship of SCN5A. Further studies are needed to elucidate the precise molecular mechanisms of PKA activation on WT-INa and V1667I-INa. The results of this study have important clinical implications. The efficacy of beta-blockers for the treatment of LQTS has so far only been proven for the LQT1 and LQT2 subtypes, with conflicting results for the LQT3 subtype (13, 14). Given the marked QT prolongation in response to epinephrine infusion in carriers of the SCN5A V1667I mutation, certain LQT3 patients may benefit from beta-blocker therapy. Future studies should clarify whether beta-blockers are effective in these patients.