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.

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.

Surgery of the left atrioventricular valve and of the left ventricular outflow tract in atrioventricular septal defect

1991 ◽  
Vol 1 (4) ◽  
pp. 344-355 ◽  
Author(s):  
Tjark Ebles
Keyword(s):  
Septal Defect ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Atrioventricular Septal Defect ◽  
Left Ventricular ◽  
Atrioventricular Valve ◽  
Left Ventricular Outflow ◽  
Cardiac Surgeon ◽  
The Individual ◽  
And Function

SummaryMalfunctioning of the left atrioventricular valve has always been, and remains, the major incremental risk factor in the repair of atrioventricular septal defect. Now that the cardiac surgeon has ample time to assess the anatomy and function of the left valve, results have improved, but are still less than ideal. On the presumption that the anterior leaflet of the mitral valve is “cleft” in this anomaly, it used to be common practice to close the “cleft”. Currently, a substantial number of surgeons employ this technique, often irrespective of the individual anatomy, and in the majority of cases with success.

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.

scholarly journals Membranous septal aneurysm in a child with Noonan syndrome and hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
PRIYADARSHINI ARUNAKUMAR ◽  
Usha MK ◽  
RAMYA S ◽  
Jayaranganath M
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Mitral Valve ◽  
Noonan Syndrome ◽  
Left Ventricular Outflow Tract ◽  
Interventricular Septum ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Mitral Leaflet ◽  
Left Ventricular Outflow

A four year old boy was diagnosed with hypertrophic cardiomyopathy with moderate degree of obstruction of the left ventricular outflow tract, secondary to asymmetric septal hypertrophy and no features of congestive heart failure, at the age of the 7 months. He was also found to have myxomatous mitral valve, systolic anterior motion of anterior mitral leaflet and moderate mitral regurgitation. There was no evidence of ventricular septal defect at this point in time.In view of phenotypic features suggestive of Noonan syndrome, he underwent genetic evaluation with target gene sequencing and was detected to have a novel heterozygous mutation in exon 13 of LZTR 1 (Leucine- zipper-like transcriptional regulator 1) gene. Echocardiogram on follow up showed increase in left ventricular outflow tract obstruction and appearance of an aneurysm in the membranous portion of interventricular septum. There were no features of right ventricular outflow obstruction, tricuspid regurgitation or aortic regurgitation. The appearance of the membranous septal aneurysm may be related to the direction of jet arising from the point of contact of the anterior mitral leaflet with the hypertrophied basal septum and hitting this part of the interventricular septum. It is an unusual mechanism for formation of membranous septal aneurysm, given its absence in the earlier echocardiograms and absence of any left to right shunt across the aneurysm. He has been initiated on beta blockers due to severe left ventricular outflow tract obstruction and is planned for septal myomectomy, resection of membranous septal aneurysm and mitral valve repair.

scholarly journals Predictors of Left Ventricular Outflow Tract Obstruction After Conventional Repair for Patients with Interrupted Aortic Arch or Coarctation of the Aorta, Combined with Ventricular Septal Defect: A Single-Center Experience

2021 ◽  
Author(s):  
Katarzyna Szaflik ◽  
Sebastian Goreczny ◽  
Katarzyna Ostrowska ◽  
Piotr Kazmierczak ◽  
Maciej Moll ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Septal Defect ◽  
Left Ventricular Outflow Tract ◽  
Primary Repair ◽  
Coarctation Of The Aorta ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Z Score ◽  
Left Ventricular Outflow

AbstractLeft ventricular outflow tract obstruction (LVOTO) affects survival and reoperation rates after surgical treatment of patients with interruption of the aortic arch (IAA) or coarctation of the aorta (CoA) with ventricular septal defect (VSD). The aim of the study was to determine predictors of LVOTO and to evaluate the relationship between aortic valve (AoV) morphology and the re-intervention rate. Retrospective review of patients, who underwent a conventional repair for IAA/CoA with VSD at a tertiary referral center between 1996 and 2017. The preoperative demographic data as well as pre- and post-operative echocardiographic parameters and re-interventions were reviewed. In the median follow-up of 8.3 years (range of 6.15–10.27) 5 patients (11.9%) from a total of 47 patients included in the study presented with a significant LVOTO. Four of them required reoperation after median period of 2.3 years (range of 0.3–7.9) after the initial surgery. Multivariable logistic regression identified AoV z-score (OR 0.44, p = 0.017) as predictor of LVOTO. The mean AoV z-score before the primary repair was significantly smaller in those with LVOTO as compared to those with unobstructed flow from the LV (− 3.58 ± 1.96 vs. − 1.44 ± 1.55; p = 0.0016). At 1-year follow-up, both groups showed an increase in the AoV z-score (p = 0.98). The re-intervention rate after primary repair (both surgical procedures and percutaneous interventions), either for LVOTO or reCoA, was higher in patients with AoV z-score ≤  − 3 (p = 0.007 vs. p = 0.46) and those, whose aortic annulus was less or equal than patient’s weight (kg) + 1.5 mm as compared to those with larger aortic annulus (p = 0.03 vs. p = 0.16). In patients after surgical treatment of IAA/CoA with VSD, the AoV z-score at diagnosis is a significant risk factor for reoperation for LVOTO. With age, AoV growth and z-score improvement is expected. Small AoV at diagnosis is correlated with increased rate of re-intervention for LVOTO and reCoA.

The Nikaidoh procedure for complex transposition of the great arteries: short-term follow-up

2016 ◽  
Vol 27 (5) ◽  
pp. 945-950
Author(s):  
Guillermo Ventosa-Fernández ◽  
Carolina Pérez-Negueruela ◽  
Javier Mayol ◽  
Marina Paradela ◽  
José M. Caffarena-Calvar
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Ventricular Outflow Tract Obstruction ◽  
Left Ventricular Outflow ◽  
Complex Forms

AbstractBackgroundThe surgical treatment for complex forms ofd-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction remains controversial. In this study, we describe the classical surgical options – namely, the Rastelli procedure and the “réparation à l’étage ventriculaire” – and present our experience with the modified Nikaidoh procedure with early and short-term follow-up results.MethodsBetween 2007 and 2014, four patients withd-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction underwent surgical repair at our institution by means of a modified Nikaidoh procedure.ResultsWith a mean follow-up of 4.5 years, survival was 100%, and none of the patients required re-intervention or mechanical circulatory support. There was no recurrence of left ventricular outflow tract obstruction and no aortic valve regurgitation classified as more than mild. Left ventricular function was preserved.ConclusionsAortic translocation with the modified Nikaidoh procedure is a safe and effective surgical treatment for certain complex forms of transposition of the great arteries, particularly those associated with ventricular septal defect and left ventricular outflow tract obstruction. It is associated with less need for re-intervention and better morbidity and mortality results in the short- and mid-term follow-up, when compared with the classical alternatives such as the Rastelli procedure.

Multiple left-sided cardiac lesions in one of Noonan's original patients

1999 ◽  
Vol 9 (6) ◽  
pp. 610-612 ◽  
Author(s):  
Jeffrey S. Danetz ◽  
Mary T. Donofrio ◽  
Richard P. Embrey
Keyword(s):  
Noonan Syndrome ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Complex Case ◽  
Review Of The Literature ◽  
Cardiac Pathology ◽  
Left Ventricular Outflow ◽  
Cardiac Lesions ◽  
Pathology Review

AbstractWe describe a complex case of obstruction of the left ventricular outflow tract in one of Dr. Noonan's original patients. Intraoperative findings revealed pathology at the valvar, subvalvar and supravalvar positions. Patients with Noonan syndrome are traditionally described as having right-sided cardiac pathology. Review of the literature revealed left-sided lesions to occur in a substantial number of these patients. We therefore suggest the routine employment of cardiac ultrasonography in all patients with Noonan syndrome with attention directed toward left-sided pathology, as well as the frequent pulmonary valvar pathology.

Outcomes Following Surgery to Address Shone Syndrome in Children

2021 ◽  
Vol 12 (3) ◽  
pp. 360-366
Author(s):  
Tyler J. Dalton ◽  
William K. Johnson ◽  
Evelyn M. Kuhn ◽  
Benjamin H. Goot ◽  
Ronald K. Woods ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Left Ventricular Outflow Tract ◽  
Heart Association ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Lv Function ◽  
Mitral Valve Stenosis ◽  
Left Ventricular Outflow

Background: Shone syndrome is characterized by coincident mitral valve stenosis and left ventricular outflow tract obstruction. Although first described in 1963, little research has expounded surgical outcomes. We sought to evaluate our experience with this cohort, emphasizing outcomes including mortality, morbidity, and cardiac function. Methods: A retrospective chart review of 46 patients who underwent operation for Shone syndrome between 1990 and May 2018 was conducted. Index operations included 32 repairs of the left ventricular outflow tract, four mitral valve repair/replacements, nine combined repairs, and one non-Shone’s repair. Median age at index procedure was 22 days (2 days-10 years). Mean follow-up was 9.1 years (2 months-21 years), and 70 additional operations (51 reoperations) were required. Three patients were lost to follow-up. Results: Overall survival was 95.7% with two late deaths. Freedom from death or transplant was 93.5%. Thirteen (28.3%) patients remained free from reoperation. Thirty-three patients required 51 reoperations of the left ventricle outflow tract (n = 12), mitral valve (n = 16), combined repairs (n = 21), and transplant (n = 1). At most recent follow-up, patients exhibited mitral stenosis (n = 21), aortic stenosis (n = 7), and diminished LV function (n = 2). Conclusion: Surgical correction of Shone’s offers excellent survival benefit, but reoperation burden is high, with >70% of patients requiring reintervention in the follow-up period. A total of 65% of patients developed recurrent obstruction of left ventricular inflow or outflow, however, ventricular function is preserved in the majority of patients. All but one patient had no functional deficits, classified as New York Heart Association I with > 60% requiring no medication.

scholarly journals Clinical and echocardiographic characteristics of patients with pathology proven left-sided non-valvular papillary fibroelastoma

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
E El-Am ◽  
A Ahmad ◽  
R Kurmann ◽  
A Sorour ◽  
M Bois ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Aortic Valve ◽  
Left Ventricle ◽  
Left Atrium ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Papillary Fibroelastoma ◽  
Left Ventricular Outflow

Abstract Funding Acknowledgements Type of funding sources: None. Background Papillary fibroelastoma (PFE) is now regarded as the most common primary tumor of the heart. Although benign, they are clinically significant for their high risk of embolization. They are most commonly found on cardiac valves but can also be present on non-valvular endocardial surfaces. The aim of this study was to better characterize patients with left-sided non-valvular PFE and its clinical sequelae. Methods We retrospectively identified patients with pathology-proven PFEs at a single center between January 1995 and December 2018 (n = 279). Patients with left-sided non-valvular PFE were analyzed. Medical records were retrospectively reviewed for clinical characteristics and outcomes. In addition, intra-operative transesophageal echocardiograms were manually reviewed to estimate overall size and location. Results During the study period, we identified 37 patients with left-sided non-valvular PFE (mean age 61 ± 14 years; 62% females) (Table). PFEs were located on the left ventricle in 41%, left atrium in 35%, and left ventricular outflow tract in 24% of patients. Around a quarter of patients (27%) had a diagnosis of hypertrophic cardiomyopathy, 19% had prior cardiac surgery, and 27% had cancer diagnosed prior to PFE diagnosis. Transient ischemic attack or stroke was the presenting symptom in 22% of patients, myocardial infarction in 6% and peripheral embolization in 6%. Median maximal length for PFE on the left ventricle was 11.1 mm [3;18], on the left atrium 9 mm [2;25], and left ventricular outflow tract 8 mm [6;13]. A minority of patients (9/37 [24%]) had associated valvular PFE on the mitral valve and/or aortic valve (1 patient had both mitral valve and aortic valve PFE, 7 had aortic valve PFE and 1 had Mitral valve PFE). Only 13 patients had follow up transthoracic/transesophageal echocardiogram 1 year after PFE removal; 4/13 (31%) had documented PFE recurrence (3 PFE recurred in the same location as the original; 1 in a different location). Conclusion Left-sided non-valvular PFE is associated with thromboembolic events and at least in those that had follow-up echocardiograms, had a high recurrence rate. More studies are needed to evaluate the management of patients with asymptomatic PFE. Abstract Figure. Baseline Characteristics

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