Ventricular Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy (Analysis Spanning 60 Years Of Practice): AJC Expert Panel.

Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on >50 years experience, surgery reliably reverses disabling heart failure by permanently abolishing mechanical outflow impedance and mitral regurgitation, with normalization of LV pressures and preserved systolic function. A consortium of 10 international currently active myectomy centers report about 11,000 operations, increasing significantly in number over the most recent 15 years. Performed in experienced multidisciplinary institutions, perioperative mortality for myectomy has declined to 0.6%, becoming one of the safest currently performed open-heart procedures. Extended myectomy relieves symptoms in >90% of patients by ≥ 1 NYHA functional class, returning most to normal daily activity, and also with a long-term survival benefit; concomitant Cox-Maze procedure can reduce the number of atrial fibrillation episodes. Surgery, preferably performed in high volume clinical environments, continues to flourish as a guideline-based and preferred high benefit: low treatment risk option for adults and children with drug refractory disabling symptoms from obstruction, despite prior challenges: higher operative mortality/skepticism in 1960s/1970s; dual-chamber pacing in 1990s, alcohol ablation in 2000s, and now introduction of novel negative inotropic drugs potentially useful for symptom management.

Survival Following Alcohol Septal Ablation or Septal Myectomy for Patients With Obstructive Hypertrophic Cardiomyopathy.

BACKGROUND: There is little information regarding long-term mortality comparing the 2 most common procedures for septal reduction for obstructive hypertrophic cardiomyopathy (HCM), alcohol septal ablation (ASA), and septal myectomy. OBJECTIVES: This study sought to compare the long-term mortality of patients with obstructive HCM following septal myectomy or ASA. METHODS: We evaluated outcomes of 3,859 patients who underwent ASA or septal myectomy in 3 specialized HCM centers. All-cause mortality was the primary endpoint of the study. RESULTS: In the study cohort, 585 (15.2%) patients underwent ASA, and 3,274 (84.8%) underwent septal myectomy. Patients undergoing ASA were significantly older (median age: 63.0 years [IQR: 52.7-72.8 years] vs 53.7 years [IQR: 44.9-62.8 years]; P < 0.001) and had smaller septal thickness (19.0 mm [IQR: 17.0-22.0 mm] vs 20.0 mm [IQR: 17.0-23.0 mm]; P = 0.007). Patients undergoing ASA also had more comorbidities, including renal failure, diabetes, hypertension, and coronary artery disease. There were 4 (0.7%) early deaths in the ASA group and 9 (0.3%) in the myectomy group. Over a median follow-up of 6.4 years (IQR: 3.6-10.2 years), the 10-year all-cause mortality rate was 26.1% in the ASA group and 8.2% in the myectomy group. After adjustment for age, sex, and comorbidities, the mortality remained greater in patients having septal reduction by ASA (HR: 1.68; 95% CI: 1.29-2.19; P < 0.001). CONCLUSIONS: In patients with obstructive hypertrophic cardiomyopathy, ASA is associated with increased long-term all-cause mortality compared with septal myectomy. This impact on survival is independent of other known factors but may be influenced by unmeasured confounding patient characteristics.

Altered intercellular communication and extracellular matrix signaling as a potential disease mechanism in human hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is considered a primary disorder of the sarcomere resulting in unexplained left ventricular hypertrophy but the paradoxical association of nonmyocyte phenotypes such as fibrosis, mitral valve anomalies and microvascular occlusion is unexplained. To understand the interplay between cardiomyocyte and nonmyocyte cell types in human HCM, single nuclei RNA-sequencing was performed on myectomy specimens from HCM patients with left ventricular outflow tract obstruction and control samples from donor hearts free of cardiovascular disease. Clustering analysis based on gene expression patterns identified a total of 34 distinct cell populations, which were classified into 10 different cell types based on marker gene expression. Differential gene expression analysis comparing HCM to Normal datasets revealed differences in sarcomere and extracellular matrix gene expression. Analysis of expressed ligand-receptor pairs across multiple cell types indicated profound alteration in HCM intercellular communication, particularly between cardiomyocytes and fibroblasts, fibroblasts and lymphocytes and involving integrin ß1 and its multiple extracellular matrix (ECM) cognate ligands. These findings provide a paradigm for how sarcomere dysfunction is associated with reduced cardiomyocyte secretion of ECM ligands, altered fibroblast ligand-receptor interactions with other cell types and increased fibroblast to lymphocyte signaling, which can further alter the ECM composition and promote nonmyocyte phenotypes.

Myocardial Bridge or Something Else?

In this E-Challenge, the authors report on a patient with symptoms of exertional dyspnea and angina, scheduled to have surgical unroofing of an identified myocardial bridge (MB). An MB is very common in patients with hypertrophic cardiomyopathy (HCM). Intraoperative transesophageal echocardiography with provocative maneuvers revealed the patient had a systolic anterior motion of the mitral valve with septal contact and resulting outflow tract obstruction despite the notable absence of significant basal septal hypertrophy. HCM has many phenotypic variants that can make the identification of patients with latent left ventricular outflow tract obstruction difficult in the absence of a high index of suspicion. In this report, the authors discuss the association between MBs and HCM and the importance of recognizing phenotypic variants of HCM.

Outcomes Over Follow-up ≥10 Years After Surgical Myectomy for Symptomatic Obstructive Hypertrophic Cardiomyopathy.

For over 50 years, surgical septal myectomy has been the preferred treatment for drug-refractory heart failure symptoms in obstructive hypertrophic cardiomyopathy (HCM). However, given the relatively youthful adult ages at which HCM surgery is usually performed, it is informative to evaluate longer-term results of myectomy after ≥10 years. We identified 139 consecutive obstructive HCM patients (50 ± 15 years of age; 55% men) who underwent surgical myectomy, 2003 to 2010 at Tufts HCM Center and followed 11.3 ± 2.7 years (range to 17). Operative mortality was low (0.6%) and left ventricular (LV) outflow gradients at rest were reduced from 56 ± 40 mm Hg preoperatively to 1 ± 7 mm Hg postoperatively, durable over the study period, with no patient requiring reoperation for the residual gradient. Over follow-up, 129 of 139 patients (93%) were alive ≥10 years after myectomy, including 17 patients ≥15 years. Of 118 patients with complete long-term clinical follow-up data, 109 (92%) experienced clinical improvement to New York Heart Association classes I or II. In 9 patients (8%) refractory class III/IV symptoms reoccurred 6.6 ± 3.9 years postoperatively, including 4 who ultimately underwent a heart transplant. After myectomy, there were 2 late HCM-related deaths, but none suddenly; notably 6 patients (12%) with prophylactic implantable cardioverter-defibrillators experienced appropriate therapy terminating ventricular tachycardia/ventricular fibrillation after myectomy. Survival following myectomy was 91% at 10 years (95% confidence interval: 85, 96%) not different from the age- and gender-matched general United States population (log-rank p = 0.64). In conclusion, myectomy provides permanent abolition of outflow gradients with reversal of heart failure and highly favorable long-term survival, representing a low-risk:high-benefit option when performed in experienced HCM centers. Myectomy did not protect absolutely against arrhythmic sudden death events, underscoring the importance of risk stratification in operative patients.

Comorbidity Burden and Adverse Outcomes After Transcatheter Aortic Valve Replacement.

Background Transcatheter aortic valve replacement (TAVR) has become the preferred treatment for symptomatic patients with aortic stenosis and elevated procedural risk. Many deaths following TAVR are because of noncardiac causes and comorbid disease burden may be a major determinant of postprocedure outcomes. The prevalence of comorbid conditions and associations with outcomes after TAVR has not been studied. Methods and Results This was a retrospective single-center study of patients treated with TAVR from January 2015 to October 2018. The association between 21 chronic conditions and short- and medium-term outcomes was assessed. A total of 341 patients underwent TAVR and had 1-year follow-up. The mean age was 81.4 (SD 8.0) years with a mean Society of Thoracic Surgeons predicted risk of mortality score of 6.7% (SD 4.8). Two hundred twenty (65%) patients had ≥4 chronic conditions present at the time of TAVR. There was modest correlation between Society of Thoracic Surgeons predicted risk of mortality and comorbid disease burden (r=0.32, P<0.001). After adjusting for Society of Thoracic Surgeons predicted risk of mortality, age, and vascular access, each additional comorbid condition was associated with increased rates of 30-day rehospitalizations (odds ratio, 1.21; 95% CI, 1.02-1.44), a composite of 30-day rehospitalization and 30-day mortality (odds ratio, 1.20; 95% CI, 1.02-1.42), and 1-year mortality (odds ratio, 1.29; 95% CI, 1.05-1.59). Conclusions Comorbid disease burden is associated with worse clinical outcomes in high-risk patients treated with TAVR. The risks associated with comorbid disease burden are not adequately captured by standard risk assessment. A systematic assessment of comorbid conditions may improve risk stratification efforts.

Plasma Proteomic Profiling in Hypertrophic Cardiomyopathy Patients before and after Surgical Myectomy Reveals Post-Procedural Reduction in Systemic Inflammation.

Left Ventricular Outflow Tract (LVOT) obstruction occurs in approximately 70% of Hypertrophic Cardiomyopathy (HCM) patients and currently requires imaging or invasive testing for diagnosis, sometimes in conjunction with provocative physiological or pharmaceutical stimuli. To identify potential biomarkers of LVOT obstruction, we performed proteomics profiling of 1305 plasma proteins in 12 HCM patients with documented LVOT obstruction, referred for surgical myectomy. Plasma was collected at the surgical preoperative visit, approximately one month prior to surgery and then at the post-surgical visit, approximately 3 months later. Proteomic profiles were generated using the aptamer-based SOMAscan assay. Principal Component Analysis using the highest statistically significant proteins separated all preoperative samples from all postoperative samples. Further analysis revealed a set of 25 proteins that distinguished the preoperative and postoperative states with a paired t-test p-value of <0.01. Ingenuity Pathway analysis facilitated the generation of protein interaction networks and the elucidation of key upstream regulators of differentially expressed proteins, such as interferon-γ, TGF-ß1, and TNF. Biological pathways affected by surgery included organ inflammation, migration, and motility of leukocytes, fibrosis, vasculogenesis, angiogenesis, acute coronary events, endothelial proliferation, eicosanoid metabolism, calcium flux, apoptosis, and morphology of the cardiovascular system. Our results indicate that surgical relief of dynamic outflow tract obstruction in HCM patients is associated with unique alterations in plasma proteomic profiles that likely reflect improvement in organ inflammation and physiological function.

The Convergent Procedure: A Unique Multidisciplinary Hybrid Treatment of Atrial Fibrillation.

The convergent procedure is a hybrid ablation treatment for atrial fibrillation. It is increasingly considered as a management option for patients with persistent and long-standing atrial fibrillation. It consists of surgical ablation of the posterior left atrium through a minimally invasive closed-chest approach followed by endocardial catheter ablation. It is increasingly performed with concurrent epicardial occlusion of the left atrial appendage with a video-assisted thoracoscopic technique to physically and electrically isolate the left atrial appendage. This article provides an overview of a multidisciplinary approach to the convergent procedure, with concurrent thoracoscopic closure of the left atrial appendage, with an emphasis on perioperative management at a single institution. It provides a literature review of procedural outcomes, current data limitations, and future considerations.

Adult Monozygotic Twins With Hypertrophic Cardiomyopathy and Identical Disease Expression and Clinical Course.

A unique clinical circumstance involving middle-aged male identical twins with obstructive hypertrophic cardiomyopathy (HC) is reported. The concordance of morphologic (i.e., phenotype) findings and clinical course between the 2 patients is remarkable, including timing of the onset and progression of heart failure due to left ventricular outflow tract obstruction, frequency of paroxysmal atrial fibrillation and beneficial response to surgical myectomy and Cox-Maze IV procedure (performed 14 days apart). Histopathology of resected ventricular septal muscle showed identical hallmarks of HC including myocyte disorganization, small vessel disease, and myocardial fibrosis. A missense variant of the CRYAB gene was identified as potentially relevant to the pathogenesis of HC in the twins. Taken together, these observations support a powerful genetic determinant for the morphologic and clinical expression of HC, with little or no environmental influence.

Right Transaxillary Transcatheter Aortic Valve Replacement Using the "Flip-n-Flex" Technique.

BACKGROUND: Right transaxillary transcatheter aortic valve replacement (TAx-TAVR) is rarely performed due to challenging vascular tortuosity and unfavorable implantation angles. We explored this procedure using the newer-generation balloon-expandable SAPIEN 3 (Edwards Lifesciences, Irvine, CA) valve and our novel "flip-n-flex" technique. The safety and effectiveness of the procedure and the learning curve of our early experiences were investigated. METHODS: The first 10 consecutive patients undergoing right TAx-TAVR performed at our center from June 2016 to May 2018 were included in the study. Patients' preoperative characteristics, procedural outcomes, and clinical outcomes were studied. Intraoperative fluoroscopy times were also reviewed to analyze the procedural learning curve. RESULTS: The 10 patients were a mean age of 81.8 ± 8.7 years, and 5 patients (50%) were women. Mean Society of Thoracic Surgeons Predicted Risk of Mortality was 12.0% ± 9.5%. Procedural success was achieved in all cases without vascular complications. Paravalvular leak was absent or mild in all patients. Two patients (20%) required permanent pacemaker implantation. The median postoperative length of stay was 4 days (range, 2-13 days). The 30-day mortality was 0%. Mean transvalvular gradient improved from 38.4 ± 12.6 mm Hg to 9 ± 4.4 mm Hg postoperatively. New York Heart Association Functional Classification improved in all patients. The fluoroscopy time showed marked reduction from 44.1 ± 8.2 minutes to 17.4 ± 2.9 minutes with the use of the flip-n-flex technique. CONCLUSIONS: Our early experience of right TAx-TAVR with the SAPIEN 3 valve demonstrated satisfactory outcomes and a quick learning as facilitated by the flip-n-flex technique. This could be a beneficial TAVR approach to suitable patients.

A case series: the outcomes, support duration, and graft function recovery after VA-ECMO use in primary graft dysfunction after heart transplantation.

Primary graft dysfunction (PGD) is a rare complication associated with high mortality after heart transplantation, which may require veno-arterial extra-corporeal membrane oxygenation (VA-ECMO) support. A standardized definition for PGD was developed by the International Society of Heart and Lung Transplantation in 2014. Due to limited reports using this definition, the detailed outcomes after VA-ECMO support remain unclear. Therefore, we retrospectively analyzed our single-center outcomes of PGD following VA-ECMO support. Between September 2014 and August 2018, 160 patients underwent heart transplantation in our single center. Nine PGD patients required VA-ECMO support, with an incidence of 5.6%. Pre-operative recipient/donor demographics, intra-operative variables, timing of VA-ECMO initiation and support duration, graft function recovery during 30 days after heart transplant, VA-ECMO complications, and survival were analyzed. The indication for VA-ECMO support was biventricular failure for all nine patients. Six patients had severe PGD requiring intra-operative VA-ECMO, while two patients had moderate PGD and one patient had mild PGD requiring post-operative VA-ECMO. All cohorts were successfully decannulated in a median of 10 days. Survival to discharge rate was 88.9%. One-year survival rate was 85.7%. Left ventricular ejection fraction recovered to normal within 30 days in all PGD patients. Our study showed VA-ECMO support led to high survival and timely graft function recovery in all cohorts. Further larger research can clarify the detailed effects of VA-ECMO support which may lead to standardized indication of VA-ECMO support for PGD patients.

Distal Embolic Protection in Impella 5.0 Explantation: Loop and Snare Technique.

The left ventricular assist device Impella 5.0 (Abiomed Inc, Danvers, MA) has become widely accepted as a temporary mechanical circulatory support for patients in cardiogenic shock. The Impella 5.0 is placed through an anastomosed graft. When removing the device, blood clot formation has been noted in the anastomosed graft. The blood clot has been reported to dislodge and embolize distally, causing acute limb ischemia. Here, we present our simple, inexpensive, and effective "loop and snare" technique for safer device removal, preventing distal embolic complications. In our experience of 6 patients who had Impella 5.0 removal with this technique, the distal embolic complication was 0%.

Intraoperative Provocative Testing in Patients with Obstructive Hypertrophic Cardiomyopathy Undergoing Septal Myectomy.

BACKGROUND: Resolution of left ventricular outflow tract (LVOT) obstruction predicts symptom relief postmyectomy. Intraoperative measurement of LVOT gradients thus is essential for surgical guidance. We hypothesized that (1) hypertrophic cardiomyopathy patients have lower LVOT gradients when measured intraoperatively with transesophageal echocardiography (TEE) compared with preoperative measurements with transthoracic echocardiography (TTE) and that (2) intraoperative provocative testing can help evaluate the adequacy of surgical resection. METHODS: We compared resting LVOT gradients on preoperative TTE to intraoperative TEE. We also compared intraoperative resting and provoked gradients pre- and postresection. Either isoproterenol 10 µg/kg/min or dobutamine 20 µg/kg/min was used. Patients with provoked LVOT gradients >30 mm Hg were considered for further resection based on LVOT/mitral valve morphology and clinical comorbidities. RESULTS: Of 315 patients identified, 293 patients were included in the analysis. There was a statistically significant difference between preoperative TTE and intraoperative TEE resting LVOT gradients (60.9 ± 39.4 mm Hg vs 42.0 ± 30.5 mm Hg, P < .0001). Out of 197 patients who had significant resting obstruction preoperatively, 82 (41.6%) demonstrated mild or no dynamic obstruction under general anesthesia. Provocative testing with both isoproterenol and dobutamine increased peak gradients (116.8 ± 33 mm Hg isoproterenol vs 107.5 ± 33 mm Hg dobutamine, P = .03). Post-cardiopulmonary bypass, seven patients (2.3%) had LVOT gradients > 30 mm Hg at rest, while 63 patients (21.5%) had residual gradients >30 mm Hg only with provocation. Elevated gradients, persistent systolic anterior motion of the mitral valve with near contact, and/or significant mitral regurgitation with provocative testing resulted in return to cardiopulmonary bypass in 41 patients (14%). CONCLUSIONS: Resting intraoperative TEE LVOT gradients are significantly lower than preoperative TTE gradients, with systolic anterior motion of the MV and outflow obstruction often not visualized after inducing general anesthesia. Intraoperative pharmacologic provocation can identify patients who may benefit from further surgical intervention, facilitating procedural success.

Efficacy of Combined Cox-Maze IV and Ventricular Septal Myectomy for Treatment of Atrial Fibrillation in Patients With Obstructive Hypertrophic Cardiomyopathy.

Atrial fibrillation (AF) has important clinical consequences in hypertrophic cardiomyopathy (HC). Safety and efficacy of the Cox-Maze IV procedure (when combined with ventricular septal myectomy) in patients with obstructive HC and paroxysmal AF is largely unresolved. Records of 395 consecutive HC patients (age 55 ± 13 years) who underwent septal myectomy for heart failure symptoms between 2004 and 2015 were reviewed. Sixty-two patients also had concomitant complete biatrial Cox-Maze IV for a history of symptomatic paroxysmal AF (3.0 ± 3.6 episodes) combined with myectomy comprise the study cohort. Freedom from symptomatic AF recurrences after operation was assessed. Left ventricular outflow gradients were reduced from 81 ± 28 mm Hg preoperatively to 1.2 ± 6.8 mm Hg after operation. At most recent follow-up, 53 patients (85%) were asymptomatic or mildly symptomatic. Freedom from recurrent symptomatic AF after myectomy/Cox-Maze IV was: 85% (95% confidence interval [CI] 73, 92) at 1 year, 69% (95% CI 55, 79) at 3 years, and 64% (95% CI 48, 75) at 5 years, including 34 patients (54%) who have experienced no symptomatic AF episodes for up to 8.2 years following surgery. The only clinical predictor of recurrent AF over follow-up was preoperative transverse left atrial dimension ≥45 mm (p <0.01). In conclusion, biatrial Cox-Maze IV combined with septal myectomy is associated with favorable long-term freedom from symptomatic paroxysmal AF recurrence, as well as from obstructive heart failure symptoms. These data support myectomy/Cox-Maze as an effective management option for the subgroup of HC patients with symptomatic outflow obstruction and paroxysmal AF.

Guideline-Based Referral for Septal Reduction Therapy in Obstructive Hypertrophic Cardiomyopathy Is Associated With Excellent Clinical Outcomes.

BACKGROUND: The outcome of medically refractory patients with obstructive hypertrophic cardiomyopathy treated according to the American College of Cardiology/American Heart Association consensus guideline recommendations is not known. The objectives of this study were to define the short- and long-term outcomes of medically refractory obstructive hypertrophic cardiomyopathy patients undergoing alcohol septal ablation (ASA) and surgical septal myectomy (SM) with patient management in accordance with these consensus guidelines, as well as to quantify procedural risk and burden of comorbid conditions at the time of treatment. METHODS AND RESULTS: Patients with obstructive hypertrophic cardiomyopathy referred for either ASA or SM from 2004 to 2015 were followed for the primary end point of short- and long-term mortality and compared with respective age- and sex-matched US populations. Of 477 consecutive severely symptomatic patients, 99 underwent ASA and 378 SM. Compared with SM, ASA patients were older ( P<0.001), had a higher burden of comorbid conditions ( P<0.01), and significantly higher predicted surgical mortality ( P<0.005). Procedure-related mortality was 0.3% and similarly low in both groups (0% in ASA and 0.8% in SM). Over 4.0±2.9 years of follow-up, 95% of patients had substantial improvement in heart failure symptoms to New York Heart Association class I/II (96% in SM and 90% in ASA). Long-term mortality was similar between the 2 groups with no difference compared with age- and sex-matched US populations. CONCLUSIONS: Guideline-based referral for ASA and SM leads to excellent outcomes with low procedural mortality, excellent long-term survival, and improvement in symptoms. These outcomes occur in ASA patients despite being an older cohort with significantly more comorbidities.

Impact of Effective Management Strategies on Patients With the Most Extreme Phenotypic Expression of Hypertrophic Cardiomyopathy.

Advances in treatment options for hypertrophic cardiomyopathy (HC) have proven effective in many patients for promoting favorable long-term outcomes. Whether this expectation is similar for patients with the most extreme expression of massive left ventricular (LV) hypertrophy, a particularly aggressive form of the disease is unresolved. Of 1,766 consecutive HC patients presenting to Tufts HC Institute (2004 to 2015), 92 were identified with extreme LV wall thickness (30 to 48 mm), and compared with 1,674 HC patients with less marked hypertrophy (13 to 29 mm). Follow-up assessment was over 5.3 ± 3.4 years. Patients with massive LV hypertrophy (n = 92) had higher sudden death event rates (3.0%/year) than did patients with lesser hypertrophy (0.8%/year; p <0.001). In 16 of the 92 patients (17%), potentially lethal ventricular tachyarrhythmia were successfully aborted by primary prevention implantable cardioverter defibrillator (ICD) therapy at 30 ± 13 years (n = 11), or by resuscitated cardiac arrest with external defibrillation (n = 5) and later by secondary prevention interventions (n = 3); no patient experienced arrhythmic sudden death. Aborted sudden death events (3.0%/year) exceeded HC-related mortality by 7-fold (n = 2; 0.4%/year; p <0.001). European Society of Cardiology risk score would have failed to identify 60% of patients with arrhythmic sudden death events, leaving them exposed to sudden death without ICDs. In addition, 35 patients required surgical myectomy for progressive heart failure due to LV outflow obstruction (improved to NYHA I/II in 30). Eighty-eight (96%) of the 92 patients have survived to age 38 ± 14 years (23% ≥ 50 years). All-cause mortality did not differ from an age and gender-matched general population (p = 0.62). In conclusion, in this referral-based population, patients with the most extreme expression of HC are at increased arrhythmic sudden death risk reliably prevented with prophylactic ICDs. Progressive heart failure secondary to outflow obstruction was reversible with surgical myectomy. Despite extreme phenotypic expression, with contemporary treatment interventions young HC patients have an opportunity to achieve extended survival with good quality of life.

Late Clinical Presentation of Prosthesis-Patient Mismatch Following Transcatheter Aortic Valve Replacement.

Prosthesis-patient mismatch (PPM) is relatively common after aortic valve replacement (AVR) and generally is associated with reduced regression of left ventricular (LV) mass. PPM after valve-in-valve transcatheter aortic valve replacement (TAVR) was reported to be 38%. PPM generally is manifested clinically by dyspnea and echocardiographically by high transvalvular gradients. In this E-Challenge, the authors will review a case of a late clinical presentation of PPM 1-year following a valve-in-valve TAVR.

Molecular Identification of Leishmania Parasites in Sand Flies (Diptera, Psychodidae) of an Endemic Foci in Poldokhtar, Iran.

BACKGROUND: Cutaneous leishmaniasis is one of the most important public health problems in many developing countries. Sand flies, as vectors, transmit infectious forms of the parasite to the vertebrate hosts. Poldokhtar, South West of Iran, is one of the endemic foci of diseases with a little information about it. In this paper, we have tried to gather some useful information to control and to prevent this disease in this region. MATERIALS AND METHODS: The present study was conducted to determine the vector(s), the parasite, and the species composition of sand flies in the Poldokhtar County during the months from July to September 2015. Sticky paper traps were used to collect sand flies from July to September. Species identification was done based on available diagnostic keys. Nested-polymerase chain reaction was performed to diagnosis the Leishmania infection of sand flies, and restriction fragment length polymorphism was used to identify the Leishmania species. RESULTS: A total of 2000 specimens comprising 8 species of sand flies (6 Phlebotomus and 2 Sergentomyia) were identified. Phlebotomus papatasi was the dominant species outdoor and Sergentomyia sintoni was the dominant species indoor. Among the 163 specimens of female P. papatasi, just 10 of them (6.1%) were positive to Leishmania major parasites. CONCLUSION: This is the first report of Leishmania infection of P. papatasi to L. major in this region. The results revealed that the high density of P. papatasi in outdoor and their infection with L. major is attributed that this species can play a major role as a principle vector in this region.

Clinical Spectrum and Management Implications of Left Ventricular Outflow Obstruction With Mild Ventricular Septal Thickness in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HC) has historically been characterized as a disease of substantial left ventricular hypertrophy, often associated with dynamic left ventricular outflow tract obstruction. However, we have recently encountered patients with subaortic obstruction and only minimal basal septal thickness, raising important management implications. Thereby, we sought to characterize the natural history and treatment strategies for this under-recognized subgroup of HC patients with dynamic obstruction. Of 1,591 consecutive patients with a HC diagnosis, 113 (7%) were identified with outflow obstruction due to elongated mitral valve leaflets producing systolic anterior motion and septal contact typical of HC (gradient, 84 ± 29 mm Hg at rest or with exercise), with maximal ventricular septal thickness ≤15 mm, including 14 patients with only 10 to 12 mm. In addition to the mechanism of outflow obstruction, other evidence supporting a HC diagnosis prominently included: positive HC family history and/or pathogenic sarcomere mutation, arrhythmic sudden death event, typical histopathology of septal muscle, and characteristic mitral valve and papillary muscle anomalies. Over 3.8 ± 3.5 years, 41 patients (36%) developed severe heart failure including 36 who have undergone myectomy associated with reconstruction of the outflow tract and mitral valve apparatus, resulting in relief of gradient without iatrogenic ventricular septal defect or mitral valve replacement. Postoperatively, all 36 patients have survived with symptom relief to New York Heart Association classes I/II. In conclusion, these observations expand the HC clinical profile and phenotype to include an under-appreciated subgroup in which disease expression includes outflow obstruction due primarily to an elongated mitral valve, associated with only minimal (or normal) ventricular septal thickness. Such HC patients can develop marked functional limitation amenable to an operative strategy that effectively relieved symptoms due to outflow obstruction, but without mitral valve replacement.