Tuberous Sclerosis Revealed by Syncope due to a Rhabdomyoma in a 1-year Old Infant

2019 ◽  
Author(s):  
Safir Soukaina
Keyword(s):  
Tuberous Sclerosis ◽  
Muscle Tone ◽  
Interventricular Septum ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Cardiac Monitoring ◽  
Pregnancy And Delivery ◽  
Left Ventricular Outflow ◽  
The Right ◽  
Pediatric Consultation

Rhabdomyoma represent a rare cardiac tumor often associated with tuberous sclerosis however is often remains asymptomatic with a regressive pattern of evolution throughout childhood. We report a rare case of an infant of 1 year old from a uneventful pregnancy and delivery,with normal developmental milestones, whose mother noticed two episodes of brief period of loss of consciousness and muscle tone upon pediatric consultation clinical examination reveal a conscious child able to stand without support with hypopigmented skin lesion a CT scan revealed periventricular calcifications with subcortical intraparenchymal hypodense plaque an EEG without signs of epileptic pattern EKG didn’t reveal signs of arrythmias and transthoracic echogardiogram showing multiple hyperechogenic homogenous tumors one attached to the interventricular septum with intermittant left ventricular outflow tract obstruction due to motion second near to the tricuspid valve with no signs of obstruction and a third attached to the free wall of the right ventricle. Tuberous sclerosis was retained as a diagnosis with rhabdomyoma and the management was based on regular cardiac monitoring with marked regression of tumor size and no recurrence of syncope episodes.

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.

scholarly journals Alcohol septal ablation in hypertrophic cardiomyopathy

2018 ◽  
Vol 2018 (3) ◽  
Author(s):  
Juan José Santos Mateo ◽  
Juan R Gimeno
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Interventricular Septum ◽  
Ventricular Outflow Tract ◽  
Clinical Results ◽  
Left Ventricular ◽  
Alcohol Septal Ablation ◽  
Obstructive Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow ◽  
Surgical Myectomy

Alcohol septal ablation (ASA) has become an alternative to surgical myectomy in obstructive hypertrophic cardiomyopathy since it was first introduced in 1994 by Sigwart. The procedure alleviates symptoms by producing a limited infarction of the upper interventricular septum, resulting in a decrease in left ventricular outflow tract (LVOT) gradient. The technique has been improved over time and the results are comparable with those of myectomy. Initial concerns about long-term outcomes have been largely resolved. In this review, we discuss indications, technical aspects, clinical results and patient selection to ASA.

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.

scholarly journals Syncope in a hypertrophic heart at a wedding party: can happiness break a thick heart? Takotsubo cardiomyopathy complicated with left ventricular outflow tract obstruction in a hypertrophic heart

2020 ◽  
Vol 2020 (6) ◽  
Author(s):  
Nicoletta Orphanou ◽  
Christos Eftychiou ◽  
Elias Papasavvas ◽  
Marios Ioannides ◽  
Panayiotis Avraamides
Keyword(s):  
Takotsubo Cardiomyopathy ◽  
Obstructive Coronary Artery Disease ◽  
Interventricular Septum ◽  
Hypertensive Heart Disease ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Interesting Case ◽  
St Segment Elevation ◽  
Left Ventricular Outflow ◽  
Sigmoid Septum

Abstract A 70-year-old woman with known history of hypertension presented because of a syncopal episode during dinner at a wedding party, followed by chest pain. On physical examination a systolic murmur was noted, and her electrocardiogram showed ST segment elevation in anterior leads. She had elevated troponin levels while echocardiography showed a hypertrophic interventricular septum with dyskinetic apex and left ventricular outflow (LVOT) obstruction. Emergency coronary angiography excluded obstructive coronary artery disease and confirmed the presence of LVOT obstruction with a gradient of 90 mm Hg. A left ventriculography showed hypercontractility of the basal and mid segments with apical wall dyskinesia indicating Takotsubo cardiomyopathy. Patient was discharged after 6 days of hospitalization with normalization of left ventricular function and regression of the LVOT obstruction. This is an interesting case of Takotsubo cardiomyopathy complicated with severe LVOT obstruction in a patient with hypertensive heart disease and a sigmoid septum hypertrophy.

scholarly journals Novel Point-of-Care Ultrasound (POCUS) Technique to Modernize the JVP Exam and Rule out Elevated Right Atrial Pressures

2021 ◽  
Author(s):  
Larry Istrail ◽  
Maria Stepanova
Keyword(s):  
Right Atrium ◽  
Point Of Care ◽  
Venous Pressure ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Right Atrial ◽  
Point Of Care Ultrasound ◽  
Status Assessment ◽  
Left Ventricular Outflow ◽  
The Right

Accurate assessment of the jugular venous pressure (JVP) and right atrial pressure (RAP) has relied on the same bedside examination method since 1930. While this technique provides a rough estimate of right-sided pressures, it is limited by poor sensitivity and overall diagnostic inaccuracy. The internal jugular vein (IJV) is difficult to visualize in many patients and relies on an incorrect assumption that the right atrium lies 5 centimeters below the sternum. Point-of-care ultrasound (POCUS) offers an alternative method for more precisely estimating JVP and RAP. We propose a novel method of measuring the right atrial depth (RAD) using a sonographic measurement of the depth of the posterior left ventricular outflow tract as a surrogate landmark to the center of the right atrium when viewed in the parasternal long axis view. This is combined with determination if JVD was present at the supraclavicular point. Sensitivity, specificity, PPV, NPV of JVD at the supraclavicular point was 70%, 76%, 59%, 91% respectively. These values were confounded by the lack of standardization of zero reference landmarks (ZRLs) used during the right heart catheterizations. When the RAD estimate was adjusted to account for measurement error the sensitivity of JVD at supraclavicular point for elevated RAP improved to 90% with negative predictive value of 96%. This may offer a rapid and reliable method for ruling out elevated RAP and increase objectivity in our volume status assessment.

scholarly journals Application of the Konno procedure for infective endocarditis in native bicuspid aortic valve with annular abscess extending into the interventricular septum

2021 ◽  
Vol 2021 (9) ◽  
Author(s):  
Toshio Doi ◽  
Kanetsugu Nagao ◽  
Hayato Obi ◽  
Akihiko Higashida ◽  
Masaya Aoki ◽  
...  
Keyword(s):  
Infective Endocarditis ◽  
Aortic Valve ◽  
Interventricular Septum ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Left Ventricular Outflow ◽  
Surgical Field ◽  
Annular Abscess ◽  
Complex Surgery ◽  
Septal Abscess

Abstract Annular abscess is a serious complication of infective endocarditis, which often requires complex surgery and has a very high post-operative mortality rate. The Konno procedure involves valve annuloplasty for a narrow aortic annulus or left ventricular outflow tract stenosis in children; it is also performed for various cardiac conditions in adults. Here, we report a case of the Konno procedure performed in a patient with aortic valve infective endocarditis, with an annular abscess extending into the interventricular septum (IVS). A 58-year-old man who presented to our hospital with fever was diagnosed with aortic valve infective endocarditis caused by Streptococcus saccharolyticus. On echocardiography, an annular abscess in the direction of the IVS was detected, and surgery was planned. The Konno procedure was performed to secure an adequate surgical field and to debride and reconstruct the cavity created by the interventricular septal abscess. The patient was discharged uneventfully 29 days after surgery.

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.

P6015Histological pattern of penetrating His bundle division: implications for atrioventricular conduction damage during and following transcatheter aortic valve implantation

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
J A Cabrera ◽  
A Porta-Sanchez ◽  
D Nunez Pernas ◽  
J M Rubio ◽  
F Navarro ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
His Bundle ◽  
Transcatheter Aortic Valve ◽  
Av Conduction ◽  
Left Ventricular Outflow ◽  
The Right ◽  
Valve Implantation ◽  
Membranous Septum

Abstract Background Severe damage to the atrioventricular conduction system is one of the most common complications of transcatheter aortic valve implantation (TAVI) and can be linked to important comorbidity, increased healthcare expense, need for long-term monitoring and pacemaker implantation. Purpose To provide a detailed description of the His bundle (HB) arrangement within the left ventricular outflow tract. Methods We examined by dissection techniques and histological sections the course of the AV conduction axis (penetrating, non-branching and branching HB) in relation with the membranous and muscular interventricular septum in 57 structurally normal human heart specimens (48 males, 77±7 years) Results The AV conduction axis is located along the inferior edge of the membranous septum (MS). The MS is divided into AV and interventricular components and is located at the base of the interleaflet triangle between the right and non-coronary leaflets of the aortic valve. The conduction axis enters the AV component of the MS and is encircled by the fibrous tissue of the central fibrous body to reach the left ventricular outflow tract. The MS showed in cadaveric hearts variable dimensions in length (4.6±1.5 mm) with a range bewteen 1 to 9mm. In 17.5% of specimens the MS length was ≤2 mm. After penetrating the AV membranous septum it has a non-branching component that in 85.5% of cases runs only for a short distance (1–3 mm) along the septal crest before giving rise to the fascicles of the left bundle (LB) on the septal surface (Type A). The most anterior fibers of the LB originate at the end of the branching portion located underneath the inferior edge of the MS. In 5 hearts (9%) the HB division was found before it reached the interventricular MS (Type B) and in 3 cases (5.5%)2–3.5 mm distal to the crest (Type C). 22 hearts (49%) were shown to have a relatively left-sided deviation of the AV bundle with the anterior part of the bundle closely related to the nadir of attachment of the right coronary leaflet of the aortic valve. In the remaining 51% of hearts, the bundle coursed centrally or with a right-sided deviation. The HB measures were (mm, mean±SD (range)): length: 3±0.6 (2–4.5), width: 3.7±1.4 (2.5–5.6), thickness: 1.4±0.5 (0.5–2.2), HB to endocardium: 0.7±0.3 (0.2–1.5). His bundle position types Conclusions A shorter membranous septum length, the variable HB dimensions and the left-sided deviation of the AV conduction axis are extremely relevant anatomic features that are linked to the frequent injury to the HB branch or complete AV block following TAVI.

Sign in / Sign up

Export Citation Format

Share Document