Sudden cardiac arrest in the field in an 18-year-old male athlete with Noonan syndrome: case presentation and 5-year follow-up

2019 ◽  
Vol 29 (09) ◽  
pp. 1214-1216
Author(s):  
Ziva Petrin ◽  
Benjamin Soffer ◽  
Steven J. Daniels
Keyword(s):  
Cardiac Arrest ◽  
Noonan Syndrome ◽  
Sudden Cardiac Arrest ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Case Presentation ◽  
Septal Thickness ◽  
Heterozygous Variant ◽  
Left Ventricular Outflow

AbstractWe present a case of sudden cardiac arrest in the field with complete neurological recovery in an 18-year-old athlete with phenotypic Noonan syndrome. Evaluation revealed interventricular septal thickness of 18 mm without left ventricular outflow tract obstruction and no other identifiable structural, electrophysiologic, or genetic abnormality except isolated heterozygous variant for desmoplakin DSP variant p.Lys2103Glu, with unknown clinical significance.

Surgical Septal Myectomy for Hypertrophic cardiomyopathy. The Iranian Experience

2021 ◽  
pp. 021849232110445
Author(s):  
Alireza Alizadeh Ghavidel ◽  
Azin Alizadehasl ◽  
Ehsan Khalilipur ◽  
Ahmadali Amirghofran ◽  
Hanieh Nezhadbahram ◽  
...  
Keyword(s):  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Septal Myectomy ◽  
Septal Thickness ◽  
Long Term Follow Up ◽  
Left Ventricular Outflow ◽  
The Mean

Introduction Hypertrophic obstructive cardiomyopathy (HOCM) is a hereditary heart muscle disorder characterized by significant myocardial hypertrophy. we assessed perioperative and long-term follow-up data of Iranian HOCM patients who underwent SM in 2 pioneering centers. Methods Clinical data of patients with HOCM septal myectomy are collected. Thirty-day outcome and long-term follow-up data for recurrence of gradient and mortality are reported. Results Ninety-six patients in two different centers enrolled in the study. Most patients of 52 patients in center 1 were male (34/52 [65.3%]).and the mean age was of 36.7  ±  19 years. Syncope before admission was reported in 5.7%, the mean left ventricular ejection fraction on admission was 53  ±  8%, the mean left ventricular outflow tract gradient was 66.3  ±  20.4 mm Hg, and the mean preoperativeseptal thickness was 25.4  ±  6.7 mm. A redo SM was performed in 3 patients (5.8%), mitral valve repair in 5 patients (9.6%), and atrioventricular repair in 5 patients (9.6%). A residual systolic anterior motion was detected in 4 patients (7.7%), the mean postoperative septal thickness was 19  ±  6 mm (25.1% septal thickness reduction), and in-hospital mortality was 5.8% (n  =  3). A longer-term follow-up showed death in 3 patients (5.8%) and late recurrent left ventricular outflow tract obstruction in 1 patient. Conclusions Transaortic myectomy is an effective surgery with acceptable early and late mortality rates. Improvements in functional status are seen in almost all patients. Appropriate SM is crucial to a good clinical outcome. Long-term survival is excellent and cardiac sudden death is extremely rare after a good surgical treatment.

Sudden death in a patient with Noonan syndrome

2010 ◽  
Vol 21 (2) ◽  
pp. 233-234
Author(s):  
Alper Aydin ◽  
Mustafa S. Yilmazer ◽  
Tayfun Gurol
Keyword(s):  
Noonan Syndrome ◽  
Left Ventricular Outflow Tract ◽  
Early Adulthood ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Structure And Function ◽  
Left Ventricular Outflow ◽  
And Function ◽  
Cardiac Manifestations

AbstractWe report here the case of a 27-year-old woman with Noonan syndrome presenting with ventricular fibrillation. After successful defibrillation, echocardiography revealed hypertrophic cardiomyopathy associated with left ventricular outflow tract obstruction. Normal echocardiographic cardiac structure and function were reported 11 years ago. This case emphasises the importance of regular follow-up in patients with congenital disorders in which cardiac manifestations might develop in early adulthood or later.

Time and age dependent decrease of NT-proBNP after septal myectomy in hypertrophic obstructive cardiomyopathy

2017 ◽  
Vol 55 (10) ◽  
Author(s):  
Hao Cui ◽  
Xi Wu ◽  
Shuiyun Wang ◽  
Bing Tang ◽  
Changsheng Zhu ◽  
...  
Keyword(s):  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Dependent Manner ◽  
Factors Affecting ◽  
Septal Myectomy ◽  
Septal Thickness ◽  
Resting Pressure ◽  
Left Ventricular Outflow

AbstractBackground:NT-proBNP level is a predictive factor in hypertrophic cardiomyopathy patients, in which left ventricular outflow tract obstruction contributes to an increased NT-proBNP level. However, studies regarding the influence of septal myectomy on NT-proBNP level in hypertrophic obstructive cardiomyopathy are lacking.Methods:One hundred and eighty-two patients who underwent septal myectomy in Fuwai Hospital from May 2011 to March 2016 and were included in the present study. Preoperative and follow-up NT-proBNP was retrospectively collected. Correlation analysis was performed to determine the factors affecting preoperative NT-proBNP and postoperative decrease.Results:The cohort had a median age of 46.2 [IQR: 36.5–53.1] years, and 106 (58.2%) patients were male. NT-proBNP decreased to 816.5 [IQR: 400.3–1661.8] pg/mL from preoperative 1732.4 [IQR: 819–3296.5] pg/mL (p<0.001). Baseline NT-proBNP was correlated with gender (p<0.001), maximum septal thickness (p<0.001), and resting pressure gradient (p=0.006). The extent of NT-proBNP decrease was positively correlated with age (p<0.001), baseline NT-proBNP (p<0.001), follow-up time (p=0.020), and enlargement of the ascending aorta (p=0.019). NT-proBNP exhibited a persistent decrease after myectomy.Conclusions:Myectomy significantly reduced NT-proBNP level in hypertrophic obstructive cardiomyopathy patients, in which a time-dependent manner of decrease indicated myocardial remodeling of the heart after myectomy.

Peritricuspid annular prostate pellet

2021 ◽  
Vol 14 (2) ◽  
pp. e238076
Author(s):  
Bryan O'Sullivan ◽  
Richard Tanner ◽  
Peter Kelly ◽  
Gerard Fahy
Keyword(s):  
Right Ventricle ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Prostate Adenocarcinoma ◽  
Left Ventricular ◽  
Premature Ventricular Contractions ◽  
Left Ventricular Outflow ◽  
Metallic Object ◽  
The Right

A 75-year-old was treated for prostate adenocarcinoma with brachytherapy in September 2018. A routine follow-up chest radiograph 3 months later revealed a metallic object of the same dimensions as a brachytherapy pellet located in the right ventricle. Further imaging showed the brachtherapy pellet was located in the anterobasal right ventricular endocardium close to the tricuspid valve. Frequent asymptomatic premature ventricular contractions were observed with likely origin from the left ventricular outflow tract, an area remote from the site of the pellet. The patient remains asymptomatic and subsequent imaging shows that the position of the pellet has not changed.

Abstract 1924: Longterm Outcome Of Pediatric Patients With Discrete Subaortic Stenosis

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Christian Drolet ◽  
Joaquim Miro ◽  
Jean-Marc Côté ◽  
Letizia Gardin ◽  
Charles V Rohlicek
Keyword(s):  
Pediatric Patients ◽  
Ventricular Outflow Tract ◽  
Aortic Insufficiency ◽  
Left Ventricular ◽  
Current Data ◽  
Subaortic Stenosis ◽  
Childhood And Adolescence ◽  
Discrete Subaortic Stenosis ◽  
Left Ventricular Outflow

INTRODUCTION: We previously reported the clinical outcomes of a large pediatric cohort with discrete subaortic stenosis (SubAS) 1 . While patients with more significant left ventricular outflow tract obstruction (LVOTO) at diagnosis progressed rapidly others with mild LVOTO at presentation showed little progression of obstruction or aortic insufficiency (AI). A limitation of our previous study was a relatively short follow-up period. OBJECTIVE: To extend the follow-up of our previously described cohort with SubAS. METHODS: Information was gathered retrospectively on 71 previously identified patients diagnosed with SubAS from 1985–1998. RESULTS: Current data was available on 61/71 patients. At diagnosis the average age was 5.2±0.5 years (mean±SEM), peak LVOT Doppler gradient (Echograd) was 30±3 mmHg, and AI (all mild) was present in 25%. At last assessment the average age was 15.7±0.4 years. Twenty patients were followed only medically and were last reassessed 9.2±1.0 years after diagnosis at 15.8±0.7 years of age. The mean Echograd was unchanged from diagnosis (19±2 vs. 19±3 mmHg). The incidence of AI on echocardiography (all mild) increased from 5% to 55% although none was detectable by auscultation. Forty-one patients underwent surgery 3.0±0.5 years after diagnosis at 7.4±0.6 years of age. The Echograd at diagnosis was 35±3 and increased to 61±6 mmHg pre-operatively. Ten patients were re-operated 5.7±0.7 years after the first surgery and 2 patients had a second re-operation. The last follow up of the operated patients occurred at 15.7±0.6 years of age. At that time the Echograd was 16±3 mmHg and the incidence of AI by echocardiography had increased to 98% (36 mild & 3 moderate) but was evident on auscultation in only 25%. CONCLUSIONS: This study represents the largest and longest contemporary follow-up of pediatric patients with discrete SubAS. Our results indicate that some patients with SubAS show significant LVOTO at diagnosis and subsequent progression. Surgery in these patients provides good relief of LVOTO but does not eliminate AI. The one third of patients presenting with mild LVOTO and AI show little progression through childhood and adolescence. We suggest that the latter can be safely followed medically. 1) Rohlicek et al. Heart 82:708;1999

U tim thai và sơ sinh: chẩn đoán trước sinh, theo dõi ba trường hợp và phẫu thuật thành công một trường hợp sau sinh tại Bệnh Viện Trung Ương Huế

2020 ◽  
Author(s):  
Du Le Ba Minh
Keyword(s):  
Heart Failure ◽  
Cardiac Tumor ◽  
Fetal Echocardiography ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Cardiac Tumors ◽  
Case Methods ◽  
Left Ventricular Outflow ◽  
Postnatal Surgery

Objective: Prenatal diagnosis, postnatal followed up of three cases of cardiac tumor and successful postnatal surgery of one case. Methods: Treatment and postnatal followed up of three cases of cardiac tumor. Those cases were diagnosed by fetal echocardiography. One of them underwent a successful postnatal surgery. Results: Three fetuses were diagnosed with fetal cardiac tumor (capable of Rhabdomyoma) at 28, 27, 38 gestational weeks. There were many tumors in one or two ventricles. These cases were referred to Hue Central Hospital for timing and location of delivery in 2019. One of them had obstruction of left ventricular outflow tract with successful surgical resection at 46 hours after birth. One of them had heart failure after birth. Evolution of three cases was favorable, heart failure regressed, cardiac tumors regressed at 7 - 9 months follow up. Conclusions: Fetal cardiac tumor can be diagnosed prenatally. Those three cases were capable of rhabdomyoma due to the presence of muliple tumors and their regression after birth. Neonatal surgical option should be reserved for the risk of cardiac flow obstruction.

scholarly journals Reversed Septal Curvature Is Associated with Elevated Troponin Level in Hypertrophic Cardiomyopathy

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Renata Rajtar-Salwa ◽  
Tomasz Tokarek ◽  
Paweł Petkow Dimitrow
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Troponin I ◽  
High Sensitivity ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Septal Thickness ◽  
Left Ventricular Outflow ◽  
Echocardiographic Parameters ◽  
Ischemic Events ◽  
Septal Curvature

The aim of study was to compare patients with hypertrophic cardiomyopathy divided according to septal configuration assessed in a 4-chamber apical window. The study group consisted of 56 consecutive patients. Reversed septal curvature (RSC) and non-RSC were diagnosed in 17 (30.4%) and 39 (69.6%) patients, respectively. Both RSC and non-RSC groups were compared in terms of the level of high-sensitivity troponin I (hs-TnI), NT-proBNP (absolute value), NT-proBNP/ULN (value normalized for sex and age), and echocardiographic parameters, including left ventricular outflow tract gradient (LVOTG). A higher level of hs-TnI was observed in RSC patients as compared to the non-RSC group (102 (29.2-214.7) vs. 8.7 (5.3-18) (ng/l), p = 0.001 ). A trend toward increased NT-proBNP value was reported in RSC patients (1279 (367.3-1186) vs. 551.7 (273-969) (pg/ml), p = 0.056 ). However, no difference in the NT-proBNP/ULN level between both groups was observed. Provocable LVOTG was higher in RSC as compared to non-RSC patients (51 (9.5-105) vs. 13.6 (7.5-31) (mmHg), p = 0.04 ). Furthermore, more patients with RSC had prognostically unfavourable increased septal thickness to left LV diameter at the end diastole ratio. Patients with RSC were associated with an increased level of hs-TnI, and the only trend observed in this group was for the higher NT-proBNP levels. RSC seems to be an alerting factor for the risk of ischemic events. Not resting but only provocable LVOTG was higher in RSC as compared to non-RSC patients.

Association between left ventricular outflow tract opening and successful resuscitation after cardiac arrest

Resuscitation ◽  
2019 ◽  
Vol 138 ◽  
pp. 8-14 ◽  
Author(s):  
Emanuele Catena ◽  
Davide Ottolina ◽  
Tommaso Fossali ◽  
Roberto Rech ◽  
Beatrice Borghi ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Successful Resuscitation ◽  
Left Ventricular Outflow

Repair of Complete Atrioventricular Septal Defects With Decellularized Extracellular Matrix: Initial and Midterm Outcomes

2017 ◽  
Vol 8 (3) ◽  
pp. 310-314 ◽  
Author(s):  
Adeel Ashfaq ◽  
Tyler Brown ◽  
Brian Reemtsen
Keyword(s):  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Single Institution ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Decellularized Extracellular Matrix ◽  
Left Ventricular Outflow ◽  
Patch Technique ◽  
Complete Atrioventricular

Objective: Since April 2010, our institution has repaired complete atrioventricular septal defects (CAVSDs) with a two-patch technique utilizing CorMatrix extracellular material. This material is potentially an attractive patch because of its theorized eventual integration with the host tissue. We sought to analyze initial outcomes of CAVSD repair with CorMatrix. Methods: Data were collected on consecutive pediatric (age <18) patients receiving two-patch CAVSD repairs with CorMatrix at a single institution from April 2010 to July 2014. Baseline and perioperative characteristics were evaluated. Echocardiograms were evaluated in both the immediate postoperative period and the most recent postoperative follow-up. Variables analyzed included left AV valve performance, residual shunting, left ventricular outflow tract (LVOT) gradient, morbidity, and mortality. Results: Fifteen patients were identified. The average age at operation was 205 days, with mean follow-up time at 1,364 days. Echocardiograms revealed the following: 12 (80%) patients showed either improved or stable left AV valve performance remaining at “mild” or less insufficiency, while two (13%) declined from “none” to mild and one (7%) from mild to “severe,” which required reoperation. There was no residual shunting or LVOT obstruction at follow-up. The single (7%) reoperation was performed after three years due to left AV valve zone of apposition dehiscence. No permanent pacemakers were needed, and no deaths were reported. Conclusion: Our initial experience with CorMatrix in the repair of CAVSD in children has resulted in good initial and midterm outcomes. The CorMatrix patch remained stable through midterm follow-up, thus may be efficacious for use in CAVSD repair.

Alcohol septal ablation in patients with severe septal hypertrophy

Heart ◽  
2019 ◽  
Vol 106 (6) ◽  
pp. 462-466 ◽  
Author(s):  
Josef Veselka ◽  
Morten Jensen ◽  
Max Liebregts ◽  
Robert M Cooper ◽  
Jaroslav Januska ◽  
...  
Keyword(s):  
Interventricular Septum ◽  
Heart Association ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Class Iii ◽  
Short Term ◽  
Alcohol Septal Ablation ◽  
Left Ventricular Outflow

ObjectiveThe current guidelines suggest alcohol septal ablation (ASA) is less effective in hypertrophic obstructive cardiomyopathy (HOCM) patients with severe left ventricular hypertrophy, despite acknowledging that systematic data are lacking. Therefore, we analysed patients in the Euro-ASA registry to test this statement.MethodsWe compared the short-term and long-term outcomes of patients with basal interventricular septum (IVS) thickness <30 mm Hg to those with ≥30 mm Hg treated using ASA in nine European centres.ResultsA total of 1519 patients (57±14 years, 49% women) with symptomatic HOCM were treated, including 67 (4.4%) patients with IVS thickness ≥30 mm. The occurrence of short-term major adverse events were similar in both groups. The mean follow-up was 5.4±4.3 years and 5.1±4.1 years, and the all-cause mortality rate was 2.57 and 2.94 deaths per 100 person-years of follow-up in the IVS <30 mm group and the IVS ≥30 mm group (p=0.047), respectively. There were no differences in dyspnoea (New York Heart Association class III/IV 12% vs 16%), residual left ventricular outflow tract gradient (16±20 vs 16±16 mm Hg) and repeated septal reduction procedures (12% vs 18%) in the IVS <30 mm group and IVS ≥30 mm group, respectively (p=NS for all).ConclusionsThe short-term results and the long-term relief of dyspnoea, residual left ventricular outflow obstruction and occurrence of repeated septal reduction procedures in patients with basal IVS ≥30 mm is similar to those with IVS <30mm. However, long-term all-cause and cardiac mortality rates are worse in the ≥30 mm group.

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