Severe Left Ventricular Outflow Tract Research Articles

The results of modified Konno procedure for complex left ventricular outflow tract obstruction

Background: Complex left ventricular outflow tract obstruction (c- LVOTO) necessitates an diagnosis, follow – up and operation to void severe left ventricular outflow tract obstruction. There are some procedures, for example: Aor–ventriculoplasty (the Konno-Rastan procedure), apicoaortic conduit… However, all procedures require a prosthetic or allogeneic valve as well as postoperative anticoagulation therapy, reoperation, or both, the modified Konno procedure which avoid that problems. Methods: This was a review of seri c-LVOTO  cases who underwent modified Konno procedure from 1/2014 to 12/2022. There were 8 patients ( 5 males and 3 females). The mean age was 5,6 years (range from 1 – 10 years) and the mean weight was 12,6  kilograms. The gradient of left ventricular outflow tract was 43 mmHg. All aortic valve were competents. Results:  The time of cardiopulmonary bypass, aortic clamping were: 76 mins; 56 mins. There was no mortality.  The gradient postopration of LVOT and RVOT were 14 mmHg and 8 mmHg. During follow-up time 61 months after surgery, there was no late death. The gradient  of LVOT and RVOT were 10 mmHg and 6 mmHg Conclusion: The modified Konno procedure had  good results with low gradient of  LVOT and RVOT

One stone, two birds: severe left ventricular outflow tract obstruction and mitral valve prolapse treated with one single ValveClamp.

One stone, two birds: severe left ventricular outflow tract obstruction and mitral valve prolapse treated with one single ValveClamp.

Abstract 17605: Multidisciplinary Team Approach in the Management of Pregnant Women With Complex Congenital Heart Disease: A Case Series

Introduction: Women with congenital heart disease (CHD) now live well into adulthood and consider pregnancy. A multidisciplinary team approach is crucial to the management of complex pregnant cardio-obstetrics patients. Here, we present a case series of three patients where careful team-based care resulted in successful delivery and without significant postpartum complications. Case series: Baseline patient characteristics and delivery details are summarized in Table 1. Patient 1, with history of repaired bicuspid aortic valve (AV) and subsequent severe bioprosthetic aortic stenosis, presented with incalcitrant supraventricular tachycardia leading to an urgent c-section. Patient 2, with history of complete Shone complex, underwent emergent c-section in the cardiac OR with extracorporeal membrane oxygenation on standby after developing rapid atrial tachycardia. Patient 3 fortunately did not suffer difficulties during her pre-planned c-section. She had a history of partial AV canal defect and subaortic membrane causing severe left ventricular outflow tract obstruction. Despite the differences between their hospitalizations and pregnancy course, utilizing a multidisciplinary approach led to no long-term postpartum complications and delivery of healthy neonates for all three patients. Discussion: Cardiovascular (CV) disease is the leading cause of maternal mortality accounting for greater than 25% of deaths. CHD is the most common CV condition encountered in pregnant women. Consistent with the Presidential Advisory from the AHA and ACOG, development of a cardio-obstetrics team is vital to managing complex maternal CV disease and minimizing CV morbidity and mortality. Conclusion: Integrating the expertise and collaboration among a multidisciplinary cardio-obstetrics team from preconception through the postpartum period allows for individualized care necessary to optimize maternal and fetal outcomes for women with complex CHD.

Abstract 17470: Comparison of Outcomes for Adult Patients With Subaortic Stenosis by Surgical or Medical Management: Single-Center Cohort Study

Introduction: Surgery is recommended subaortic stenosis (SAS) patients with severe left ventricular outflow tract obstruction and symptoms, left ventricular dysfunction and sometimes aortic regurgitation, although how surgery modifies patient prognosis is not well understood. We compared the outcomes of SAS based on past and follow-up treatment disposition. Methods: This is a retrospective observation study of adult patients (≥ 18 years) evaluated for SAS on transthoracic echocardiography at our institution during 1/1/2001 to 6/30/2022. Patients were divided into three groups: 1. Patients with prior SAS surgery at index visit; 2. Patients who underwent surgery during follow-up without prior SAS surgery; and 3. Patients managed medically. Primary follow-up endpoint all-cause mortality and secondary endpoint heart failure (HF) hospitalization were compared between groups. Results: There were a total of 115, 106, and 263 patients in group 1, 2, and 3 respectively. The mean age was 45.7±20 years and 67.1% were females. Over median follow-up of 5.5 years, there were 8, 6 and 42 deaths, 3, 9 and 21 heart failure hospitalization, and 20, 15, and 25 SAS recurrences in groups 1, 2, and 3, respectively. Multivariate analysis showed that patients with shortness of breath (odds ratio 3.64, 95% confidence interval 1.20-11.05, P=0.02) and those who are treated medically (1.77 (1.03-3.05), P=0.03) are associated with higher rates of composite death and HF hospitalization. The figure illustrates the Kaplan-Meier survival curves for the primary and secondary endpoint. Group 3 had significantly worse all-cause mortality (P=0.04) and heart failure hospitalization (P=0.03) rates during follow-up. Conclusions: The cohort of adult SAS patients that were managed medically were associated with worse prognosis.

Outcomes of Alcohol Septal Ablation in Patients With Severe Left Ventricular Outflow Tract Obstruction: A Propensity Score Matching Analysis.

The current ACC/AHA guidelines on hypertrophic cardiomyopathy (HCM) caution that alcohol septal ablation (ASA) might be less effective in patients with left ventricular outflow tract obstruction (LVOTO) ≥ 100 mm Hg. We used a multinational registry to evaluate the outcome of ASA patients according to baseline LVOTO. A total of 1346 ASA patients were enrolled and followed for 5.8 ± 4.7 years (7764 patient-years). The patients with baseline LVOTO ≥ 100 mm Hg were significantly older (61 ± 14 years vs 57 ± 13 years; P < 0.01), more often women (60% vs 45%; P < 0.01), and had a more pronounced HCM phenotype than those with baseline LVOTO < 100 mm Hg. There were no significant differences in the occurrences of 30-day major cardiovascular adverse events in the 2 groups. After propensity score matching (2 groups, 257 pairs of patients), the long-term survival was similar in both groups (P= 0.10), the relative reduction of LVOTO was higher in the group with baseline LVOTO ≥ 100 mm Hg (82 ± 21% vs 73 ± 26%; P < 0.01), but the residual resting LVOTO remained higher in this group (23 ± 29 mm Hg vs 13 ± 13 mm Hg; P < 0.01). Dyspnoea (NYHA functional class) at the most recent clinical check-up was similar in the 2 groups (1.7 ± 0.7 vs 1.7 ± 0.7; P= 0.85), and patients with baseline LVOTO ≥ 100 mm Hg underwent more reinterventions (P= 0.02). After propensity matching, ASA patients with baseline LVOTO ≥ 100 mm Hg had similar survival and dyspnoea as patients with baseline LVOTO < 100 mm Hg, but their residual LVOTO and risk of repeated procedures were higher.

1-Year Outcomes Following Transfemoral Transseptal Transcatheter Mitral Valve Replacement: Intrepid TMVR Early Feasibility Study Results

BackgroundHigh surgical risk may preclude mitral valve replacement in many patients. Transcatheter mitral valve replacement (TMVR) using transfemoral transseptal access is a novel technology for the treatment of mitral regurgitation (MR) in high-risk surgical patients. ObjectivesThis analysis evaluates 30-day and 1-year outcomes of the Intrepid TMVR Early Feasibility Study in patients with ≥moderate-severe MR. MethodsThe Intrepid TMVR Early Feasibility Study is a multicenter, prospective, single-arm study. Clinical events were adjudicated by a clinical events committee; endpoints were defined according to Mitral Valve Academic Research Consortium criteria. ResultsA total of 33 patients, enrolled at 9 U.S. sites between February 2020 and August 2022, were included. The median age was 80 years, 63.6% of patients were men, and mean Society of Thoracic Surgeons Predicted Risk of Mortality for mitral valve replacement was 5.3%. Thirty-one (93.9%) patients were successfully implanted. Median postprocedural hospitalization length of stay was 5 days, and 87.9% of patients were discharged to home. At 30 days, there were no deaths or strokes, 8 (24.2%) patients had major vascular complications and none required surgical intervention, there were 4 cases of venous thromboembolism all successfully treated without sequelae, and 1 patient had mitral valve reintervention for severe left ventricular outflow tract obstruction. At 1 year, the Kaplan-Meier all-cause mortality rate was 6.7%, echocardiography showed ≤mild valvular MR, there was no/trace paravalvular leak in all patients, median mitral valve mean gradient was 4.6 mm Hg (Q1-Q3: 3.9-5.3 mm Hg), and 91.7% of survivors were in NYHA functional class I/II with a median 11.4-point improvement in Kansas City Cardiomyopathy Questionnaire overall summary scores. ConclusionsThe early benefits of the Intrepid transfemoral transseptal TMVR system were maintained up to 1 year with low mortality, low reintervention, and near complete elimination of MR, demonstrating a favorable safety profile and durable valve function.

Acute Left Ventricular Ballooning: Tools to Differentiate Hypertrophic Cardiomyopathy with Outflow Obstruction from Neurohumoral Takotsubo Syndrome

Despite considerable interest in the syndrome of acute left ventricular (LV) ballooning, its pathophysiology has remained ill-defined. In this review, we explore observational data describing two etiologies of acute LV ballooning: neurohumoral classic Takotsubo Syndrome (TTS), and acute severe unrelenting left ventricular outflow tract (LVOT) obstruction in patients with obstructive hypertrophic cardiomyopathy (HCM). We describe the clinical presentation and varying pathophysiology of these presentations, explore how echocardiography and cardiac catheterization may help differentiate between the two etiologies, and detail differences in management. We highlight the significant overlap as well as key differentiating features of these conditions, with the aim to improve diagnostic awareness and accuracy and appropriately tailor therapy.

Hypertrophic Obstructive Cardiomyopathy Associated with Moderate Aortic Stenosis in Symptomatic 75-Year-Old Female: A Case Report

A 72-year-old female was found to have symptomatic hypertrophic obstructive cardiomyopathy associated with significant aortic stenosis. She was found to have severe left ventricular outflow tract obstruction, asymmetric septal hypertrophy, significant systolic anterior motion of the anterior mitral valve leaflet, and mitral regurgitation. She was also found to have moderate aortic stenosis that made the obstruction even worse which made the patient progressively symptomatic not responding to medical therapy. She eventually underwent cardiac surgery in form of left ventricular septal myomectomy and bioprosthetic aortic valve replacement (tissue AVR) with a size 23 tissue valve, aortic root enlargement with bovine pericardial patch. She is currently stable on optimal medical therapy, feels much better and stable cardiac-wise with much better quality of life.

Prevalence and Prognosis of Atenolol-Responsive Systolic Anterior Motion of the Septal Mitral Valve Leaflet in Young Cats with Severe Dynamic Left Ventricular Outflow Tract Obstruction.

Severe dynamic left ventricular outflow tract obstruction (DLVOTO) secondary to the systolic anterior motion of the septal mitral valve leaflet (SAM) can result either from congenital mitral valve disorders or left ventricular concentric hypertrophy of any cause, in cats commonly hypertrophic cardiomyopathy (HCM). Though HCM cannot be reversed, the question remains whether atenolol can cure cats with severe DLVOTO resulting from a presumed mitral valve dysplasia. In this retrospective case series, client-owned asymptomatic cats younger than 1.5 years with echocardiographic evidence of SAM and severe DLVOTO were included. Oral atenolol therapy and recheck echocardiography after 2-3 months were recommended. The owners and referring veterinarians were called for long-term follow-up information. Of the 28 included cats, 23 were treated with atenolol. Recheck echocardiography performed in 17 cats showed a resolution of SAM in 47%. In the long term, SAM remained absent in only 9% of the treated cats. Cardiac-related death occurred in 26% of the atenolol-treated cats. The long term benefit of twice-daily atenolol therapy was documented in 9% of cats. Whether the cats where atenolol failed to resolve DLVOTO on long-term had HCM, or a therapy-resistant congenital primary mitral valve disorder remains unclear.

Impact of moderate or severe left ventricular outflow tract calcification on clinical outcomes of patients with severe aortic stenosis undergoing transcatheter aortic valve implantation with self- and balloon-expandable valves: a post hoc analysis from the SOLVE-TAVI trial.

Left ventricular outflow tract (LVOT) calcification has been associated with worse outcomes in patients undergoing transcatheter aortic valve implantation (TAVI) and may influence the selection of prosthetic valve type. We aimed to evaluate the impact of LVOT calcification on outcomes after TAVI with a self-expanding valve (SEV) versus a balloon-expandable valve (BEV). Patients of the SOLVE-TAVI trial, randomised to Edwards SAPIEN 3 or Medtronic Evolut R, were divided according to LVOT calcification into no/mild (≤1 calcium nodule extending <5 mm and covering <10% of the LVOT perimeter) and moderate/severe LVOT calcification groups. The primary endpoint was a composite of death, stroke, moderate/severe paravalvular regurgitation, permanent pacemaker implantation and annulus rupture at 30 days. Additional endpoints included all-cause and cardiovascular mortality at 1 year. Out of 416 eligible patients, moderate/severe LVOT calcification was present in 143 (34.4%). Moderate/severe LVOT calcification was associated with significantly longer fluoroscopy time and higher rates of pre- and post-dilation. Regardless of the LVOT calcification group, there was no significant difference in the primary endpoint associated with the valve type (no/mild LVOT calcification group: SEV 25.0% vs BEV 27.0%; hazard ratio [HR] 1.10, 95% confidence interval [95% CI]: 0.68-1.73; p=0.73 and moderate/severe LVOT calcification group: SEV 25.0% vs BEV 19.4%; HR 0.76, 95% CI: 0.38-1.61; p=0.49), no significant interaction between LVOT calcification and valve type (pint=0.29) and no differences between SEV vs BEV within LVOT calcification groups regarding 1-year all-cause and cardiovascular mortality. Moderate/severe LVOT calcification was associated with longer fluoroscopy time and an increased need for pre- and post-dilation, but not with a higher incidence of early and mid-term adverse clinical outcomes, regardless of valve type. (ClinicalTrials.gov: NCT02737150).

Ross procedure in neonates and infants: A valuable operation with defined limits

ObjectiveThe Ross procedure is an important tool that offers autologous tissue repair for severe left ventricular outflow tract (LVOT) pathology. Previous reports show that risk of mortality is highest among neonates and infants. We analyzed our institutional experience within this patient cohort to identify factors that most affect clinical outcome. MethodsA retrospective chart review identified all Ross operations in neonates and infants at our institution over 27 years. The entire study population was analyzed to determine risk factors for mortality and define outcomes for survival and reintervention. ResultsFifty-eight patients underwent a Ross operation at a median age of 63 (range, 9-156) days. Eighteen (31%) were neonates. Eleven (19%) patients died before hospital discharge. Multiple regression analysis of the entire cohort identified young age (hazard ratio [HR], 1.037; P = .0045), Shone complex (HR, 17.637; P = .009), and interrupted aortic arch with ventricular septal defect (HR, 16.01; P = .031) as independent predictors of in-hospital mortality. Receiver operating characteristic analysis (area under the curve, 0.752) indicated age younger than 84 days to be the inflection point at which mortality risk increases. Of the 47 survivors, there were 2 late deaths with a mean follow-up of 6.7 (range, 2.1-13.1) years. Three patients (6%) required LVOT reintervention at 3, 8, and 17.5 years, respectively, and 26 (55%) underwent right ventricular outflow tract reintervention at a median of 6 (range, 2.5-10.3) years. ConclusionsRoss procedure is effective in children less than one year of age with left sided obstructive disease isolated to the aortic valve and/or aortic arch. Patients less than 3 months of age with Shone or IAA/VSD are at higher risk for morbidity and mortality. Survivors experience excellent intermediate-term freedom from LVOT reintervention.

Hypertrophic obstructive cardiomyopathy complicated with acute myocardial infarction and diffuse fibrosis: surgery or not?

BackgroundHypertrophic cardiomyopathy with extreme hypertrophy, biventricular obstruction and diffuse myocardial fibrosis complicated by myocardial infarction in the absence of obstructive coronary artery disease (MINOCA) is a rare phenotype. Evidence and guideline recommendations are still lacking for a treatment strategy.Case presentationEmergency coronary angiography was performed in a 38-year-old man with a 2-year history of nonobstructive hypertrophic cardiomyopathy (HCM) presenting with acute myocardial infarction. The coronary angiogram yielded no stenotic lesions but showed a diffusely dilated left descending artery with slow blood flow. All evidence from biomarker analysis, electrocardiography, echocardiography, and imaging supported the diagnosis of acute myocardial infarction in the left ventricular anterior wall. The echocardiogram demonstrated severe interventricular and apical hypertrophy, severe left ventricular outflow tract obstruction and mild right ventricular outflow tract obstruction. Cardiac magnetic resonance imaging showed a concentric morphological subtype of HCM with diffuse late gadolinium enhancement in the left ventricle. Extended septal myectomy was performed 1 month later, and the patient recovered well.ConclusionsHypertrophic obstructive cardiomyopathy with acute myocardial infarction is an indication for coronary angiography. Septal reduction surgery could be performed cautiously in HCM patients with extreme hypertrophy, biventricular obstruction and diffuse myocardial fibrosis complicated by MINOCA to improve the patient’s symptoms.

Study design and rationale of VALOR-HCM: evaluation of mavacamten in adults with symptomatic obstructive hypertrophic cardiomyopathy who are eligible for septal reduction therapy

BackgroundHypertrophic cardiomyopathy (HCM) is a primary myocardial disorder which frequently leads to symptoms such as dyspnea and exercise intolerance, often due to severe dynamic left ventricular outflow tract obstruction (LVOTO). Current guideline-recommended pharmacotherapies have variable therapeutic responses to relieve LVOTO. In recent phases 2 and 3, clinical trials for symptomatic obstructive HCM (oHCM), mavacamten, a small molecule inhibitor of β-cardiac myosin has been shown to improve symptoms, exercise capacity, health status, reduce LVOTO, along with having a beneficial impact on cardiac structure and function. MethodsVALOR-HCM is designed as a multicenter (approximately 20 centers in United States) phase 3, double-blind, placebo-controlled, randomized study. The study population consists of approximately 100 patients (≥18 years old) with symptomatic oHCM who meet 2011 American College of Cardiology/American Heart Association and/or 2014 European Society of Cardiology HCM-guideline criteria and are eligible and willing to undergo septal reduction therapy (SRT). The study duration will be up to 138 weeks, including an initial 2-week screening period, followed by16 weeks of placebo-controlled treatment, 16 weeks of active blinded treatment, 96 weeks of long-term extension, and an 8-week posttreatment follow-up visit. The primary endpoint will be a composite of the decision to proceed with SRT prior to or at Week 16 or remain guideline eligible for SRT at Week 16. Secondary efficacy endpoints will include change (from baseline to Week 16 in the mavacamten group vs placebo) in postexercise LVOT gradient, New York Heart Association class, Kansas City Cardiomyopathy Questionnaire clinical summary score, NT-proBNP, and cardiac troponin. Exploratory endpoints aim to characterize the effect of mavacamten on multiple aspects of oHCM pathophysiology. ConclusionsIn severely symptomatic drug-refractory oHCM patients meeting guideline criteria of eligibility for SRT, VALOR-HCM will primarily study if a 16-week course of mavacamten reduces or obviates the need for SRT using clinically driven endpoints.

Is it Possible to Bring 4 Pregnancies to Term with Obstructive HCM without Maternal or Fetal Complications? (A Case Report and Literature Review)

Obstructive hypertrophic cardiomyopathy (HCM) during pregnancy is associated with significant maternal and perinatal morbidity. Physiological changes during pregnancy can induce complications: sudden death, heart failure and arrhythmia involving the maternal and fetal prognosis. Women with HCM generally tolerate pregnancy well. The risk is however higher in women who are symptomatic before pregnancy or in those with severe left ventricular outflow tract obstruction. The incidence of arrhythmias does not appear to be increased during pregnancy and maternal mortality is low. Prior to conception, women with HCM should have a risk assessment as well as genetic counseling. A vaginal delivery with regional anesthesia is usually appropriate. Women should be managed by a specialist multidisciplinary team.

Minimally Invasive Surgical Resection of Accessory Mitral Valve Tissue Causing Severe Symptomatic Left Ventricular Outflow Tract Obstruction in an Adult

Objective: We report a rare case of adult onset, symptomatic, severe left ventricular outflow tract obstruction secondary to accessory mitral valve tissue that was treated surgically through a minimally invasive partial sternotomy approach. Methods: A 29-year-old female with a previous diagnosis of a sub-aortic membrane with 6 months of progressive chest pain, fatigue, palpitations, and presyncope presented to the emergency department with acute onset of severe chest pain, left arm numbness, palpitations, and light-headedness. Transthoracic and subsequent transesophageal echocardiogram demonstrated a mobile, parachute-like mass in the left ventricular outflow track with severe outflow obstruction (resting velocity 3.9m/s and peak gradient 60mmHg). The patient was transferred to our institution for surgical evaluation. Results: Review of the echocardiographic imaging demonstrated obstructing accessory mitral valve tissue (Figure A). The patient underwent surgical resection of the obstructing mass through a partial upper-sternotomy. Accessory mitral valve tissue attached to the aorto-mitral curtain was removed en bloc with chordal attachments to the posteromedial papillary muscle and muscular interventricular septum (Figure C and D). A small, hemodynamically insignificant sub-aortic membrane was also excised. There was no residual gradient, and valvular and ventricular functions were preserved (Figure B). The patient was discharged home on postoperative day 3. Conclusions: While accessory mitral valve tissue rarely presents in adulthood it should remain part of the differential diagnosis of symptomatic left ventricular outflow tract obstruction. Early surgical intervention is recommended and may be performed safely via less invasive surgical approaches. Figure. Accessory mitral valve tissue causing symptomatic, severe left ventricular outflow tract obstruction before (A) and after (B) surgical resection though a minimally invasive partial sternotomy. (C) Surgeon’s view of the left ventricular outflow tract through the aortic valve of the accessory mitral valve tissue, with severed chordae tendinae, attached to the aorto-mitral curtain beneath the left/non-coronary commissure of the aortic valve. (D) Resected accessory mitral valve tissue

Echocardiographic Characteristics of Severe Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

It is important to diagnose severe left ventricular outflow tract (LVOT) obstruction in symptomatic patients with hypertrophic cardiomyopathy (HCM) to determine who might benefit from septal reduction therapy. Due to its dynamic nature, LVOT obstruction may not be present at rest, 1 Maron M.S. Olivotto I. Betocchi S. Casey S.A. Lesser J.R. Losi M.A. et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. New Engl J Med. 2003; 348: 295-303 Crossref PubMed Scopus (1012) Google Scholar , 2 Maron M.S. Olivotto I. Zenovich A.G. Link M.S. Pandian N.G. Kuvin J.T. et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation. 2006; 114: 2232-2239 Crossref PubMed Scopus (650) Google Scholar , 3 Ayoub C. Geske J.B. Larsen C.M. Scott C.G. Klarich K.W. Pellikka P.A. Comparison of Valsalva maneuver, amyl nitrite, and exercise echocardiography to demonstrate latent left ventricular outflow obstruction in hypertrophic cardiomyopathy. Am J Cardiol. 2017; 120: 2265-2271 Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar and it is then necessary to “search for obstruction” with further provocative testing. 4 Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al. 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2020; 76: e159-e240 Crossref PubMed Scopus (76) Google Scholar , 5 Geske J.B. Sorajja P. Ommen S.R. Nishimura R.A. Left ventricular outflow tract gradient variability in hypertrophic cardiomyopathy. Clin Cardiol. 2009; 32: 397-402 Crossref PubMed Scopus (54) Google Scholar , 6 Nishimura R.A. Ommen S.R. Hypertrophic cardiomyopathy: the search for obstruction. Circulation. 2006; 114: 2200-2202 Crossref PubMed Scopus (41) Google Scholar We sought to determine whether there are resting echocardiographic characteristics associated with latent LVOT obstruction.

Everolimus treatment in a 3-month-old infant with tuberous sclerosis complex cardiac rhabdomyoma, severe left ventricular outflow tract obstruction, and hearing loss.

Tuberous sclerosis complex is a rare multisystem genetic disorder characterised by the growth of numerous tumour-like malformations in many parts of the body including skin, kidneys, brain, lung, eyes, liver, and heart. Mutations in the TSC1 or TSC2 genes have been reported to cause disruption in the TSC1-TSC2 intracellular protein complex, causing over-activation of the mammalian target of rapamycin protein complex. In this study, we present a 3-month-old male infant diagnosed with tuberous sclerosis, bilateral neurosensorial hearing loss, Wolff-Parkinson-White syndrome on electrocardiography, multiple cardiac rhabdomyomas with severe stenosis in the left ventricular outflow tract, who responded well to the Everolimus therapy.

Asymptomatic patients with severe hypertrophic obstructive cardiomyopathy: New perspectives on early percutaneous treatment

Key Points Hypertrophic cardiomyopathy is the most common genetic cardiac disease. Reverse cardiac remodeling after alcohol septal ablation has been reported in patients with obstructive hypertrophic cardiomyopathy who are symptomatic despite medical therapy. No data exist on early percutaneous therapy to reverse adverse remodeling and improve prognosis in asymptomatic patients with severe left ventricular outflow tract obstruction.

An unusual neonatal case of reverse flow in the aortic arch and persistent pulmonary hypertension

The reverse flow in the aortic arch in newborn is a rare finding due to congenital heart disease with blood "stealing" from the descending aorta or severe left ventricular outflow tract obstruction and to extracardiac abnormalities as systemic arteriovenous malformation. We describe a female newborn with unexpected reverse flow in the aortic arch due to severe persistent pulmonary hypertension (PPH). The presence of reverse flow in the aortic arch in PPH, secondary to compromised LV output, was rarely reported and investigated but can be a helpful sign of worse clinical course and poor prognosis.

The Pregnancy Heart Team Approach for the Adult with Hypertrophic Cardiomyopathy and Severe Left Ventricular Outflow Tract Obstruction

Abstract Hypertrophic cardiomyopathy (HCM), the most common single-gene cardiovascular disease, is associated with increased risk for arrhythmias, heart failure, and sudden cardiac death. The hemodynamic changes known to occur during pregnancy can exacerbate heart failure and arrhythmias in women with HCM. We present a 30-year-old woman with HCM to illustrate the benefits of multidisciplinary team management of severe left ventricular outflow tract obstruction (peak gradient &gt;100 mmHg) for optimal maternal and fetal outcomes.