scholarly journals Unusual presentations of ‘‘left ventricular non compaction’’- A rare form of cardiomyopathy

2021 ◽  
Vol 9 (2) ◽  
pp. 119-124
Author(s):  
Maheen S ◽  
Srikala MJ
Keyword(s):  
Diagnostic Imaging ◽  
Ischemic Cardiomyopathy ◽  
Clinical Presentation ◽  
Case Series ◽  
Paediatric Population ◽  
Left Ventricular ◽  
Rare Form ◽  
True Prevalence ◽  
Imaging Pitfalls

Left ventricular non-compaction is an overall rare form of non-ischemic cardiomyopathy. The true prevalence of left ventricular non compaction (LVNC) is unclear, but it has been reported in 0.014–0.05% of adults and even less in paediatric population [1]. We present the case series of the patients who presented with varied clinical presentation and diagnosed to have left ventricular non-compaction on imaging. We have reviewed the literature regarding diagnostic imaging criteria and imaging pitfalls of this condition.

scholarly journals Ventricular tachycardia triggered by pregnancy in left-ventricular non-compaction cardiomyopathy: a controversial indication to automated defibrillator implantation

2020 ◽  
Vol 90 (3) ◽  
Author(s):  
Elisa Gherbesi ◽  
Carla Bonanomi ◽  
Vera Bottari ◽  
Gian Battista Danzi
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Ventricular Arrhythmias ◽  
Clinical Presentation ◽  
Left Ventricular ◽  
Rare Form ◽  
Long Term Follow Up ◽  
Malignant Arrhythmias

Left-ventricular non-compaction (LVNC) is a rare form of cardiomyopathy. Its clinical presentation is highly variable and during pregnancy is frequently associated with heart failure, embolic events, and arrhythmias. Herein we report a case of a woman with left ventricular non-compaction who had an automated defibrillator implantation for recurrent ventricular arrhythmias during pregnancy. During pregnancy and at long-term follow-up no interventions of the device were documented. In conclusion, the management of malignant arrhythmias during pregnancy is one of the concerns for patients with LVNC and requires a careful approach in third-level centers.

Aortico-left ventricular tunnel and left ventricular non-compaction: a case series

2015 ◽  
Vol 26 (2) ◽  
pp. 382-385 ◽  
Author(s):  
Brandon M. Smith ◽  
Clinton D. Cochran ◽  
Sonal T. Owens
Keyword(s):  
Medical Literature ◽  
Clinical Presentation ◽  
Case Series ◽  
Left Ventricular ◽  
Limited Information ◽  
Cardiac Anomalies ◽  
Clinical Presentations ◽  
Congenital Cardiac Anomalies

AbstractAortico-left ventricular tunnel and left ventricular non-compaction are rare congenital cardiac anomalies with varied clinical presentations and sparsely described co-existence in the medical literature. Owing to the limited information about these diagnoses in tandem, we present four cases of aortico-left ventricular tunnel and left ventricular non-compaction from our institution and discuss the clinical presentation, management, and follow-up.

A dreadful sign of aortic dissection: Aortic intimo-intimal intussusception with prominent intimal flap prolapsing into the left ventricular apex

VASA ◽  
2009 ◽  
Vol 38 (2) ◽  
pp. 181-184 ◽  
Author(s):  
Ozer ◽  
Davutoglu ◽  
Burma ◽  
Sucu ◽  
Sarı
Keyword(s):  
Aortic Dissection ◽  
Clinical Presentation ◽  
Left Ventricular ◽  
Left Ventricular Cavity ◽  
Acute Type ◽  
Clinical Form ◽  
Intimal Flap ◽  
Acute Type A Dissection ◽  
Type A Dissection ◽  
Intimal Intussusception

Intimo-intimal intussusception is an unusual clinical form of aortic dissection resulting from circumferential detachment of the intima. Clinical presentation varies according to the level of detached intima in the aorta. We present a case of acute type A dissection with prominent prolapse of the circumferential detachment intimal flap into the left ventricular cavity extended to the apex.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

scholarly journals Cardiovascular involvement in alpha-n-acetyl neuraminidase deficiency syndromes (sialidosis type I and II)

2021 ◽  
pp. 1-3
Author(s):  
Priyanka Prasanna ◽  
Chenni S. Sriram ◽  
Sarah H. Rodriguez ◽  
Utkarsh Kohli
Keyword(s):  
Autosomal Recessive ◽  
Ventricular Dysfunction ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Rare Condition ◽  
Autosomal Recessive Disorder ◽  
Left Ventricular ◽  
Type I ◽  
Neonatal Onset ◽  
Cardiovascular Involvement

Abstract Sialidosis, a rare autosomal recessive disorder, is caused by a deficiency of NEU1 encoded enzyme alpha-N-acetyl neuraminidase. We report a premature male with neonatal-onset type II sialidosis which was associated with left ventricular dysfunction. The clinical presentation and subsequent progression which culminated in his untimely death at 16 months of age are succinctly described. Early-onset cardiovascular involvement as noted in this patient is not well characterised. The case report is supplemented by a comprehensive review of the determinants, characteristics, and the clinical course of cardiovascular involvement in this rare condition.

scholarly journals Efficient implementation of the ‘non-biopsy approach’ for the diagnosis of childhood celiac disease in the Netherlands: a national prospective evaluation 2010–2013

2021 ◽  
Author(s):  
Caroline R. Meijer ◽  
Joachim J. Schweizer ◽  
Anne Peeters ◽  
Hein Putter ◽  
M. Luisa Mearin
Keyword(s):  
Celiac Disease ◽  
The Netherlands ◽  
Clinical Presentation ◽  
Paediatric Population ◽  
Hla Typing ◽  
Newly Diagnosed ◽  
Prospective Evaluation ◽  
Espghan Guidelines ◽  
Intestinal Symptoms ◽  
Nationwide Implementation

AbstractThe aim of this study was (1) to prospectively evaluate the nationwide implementation of the ESPGHAN-guidelines for the diagnosis of celiac disease (CD), (2) to investigate the incidence and clinical presentation of diagnosed childhood CD (0–14 years) in the Netherlands, and (3) to compare the findings with national survey data from 1975 to 1990 and 1993 to 2000 using the same approach. From 2010 to 2013, all practicing paediatricians were invited to report new celiac diagnoses to the Dutch Pediatric Surveillance Unit. Data were collected via questionnaires. A total of 1107 children with newly diagnosed CD were reported (mean age, 5.8 years; range, 10 months–14.9 years; 60.5% female). After the introduction of the non-biopsy approach in 2012, 75% of the diagnoses were made according to the guideline with a significant decrease of 46.3% in biopsies. The use of EMA and HLA-typing significantly increased with 25.8% and 62.1%, respectively. The overall incidence rate of childhood CD was 8.8-fold higher than in 1975–1990 and 2.0-fold higher than in 1993–2000. During the study period, the prevalence of diagnosed CD was 0.14%, far below 0.7% of CD identified via screening in the general Dutch paediatric population. Clinical presentation has shifted towards less severe and extra-intestinal symptoms.Conclusion: ESPGHAN guidelines for CD diagnosis in children were effectively and rapidly implemented in the Netherlands. Incidence of diagnosed CD among children is still significantly rising with a continuous changing clinical presentation. Despite the increasing incidence of diagnoses, significant underdiagnosis still remains. What is Known:• Since 2000 the incidence of diagnosed childhood CD in the Netherlands has shown a steady rise.• The rise in incidence has been accompanied by a changing clinical presentation at diagnosis. What is New:• The ESPGHAN guidelines 2012 for CD diagnosis were effectively and rapidly implemented in the Netherlands.• The incidence of diagnosed childhood CD in the Netherlands has continued to rise significantly during the reported period.

scholarly journals Thigh intramuscular fat predicts the prognosis in patients in non-ischemic cardiomyopathy with reduced ejection fraction

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Yoshida ◽  
A Shibata ◽  
A Tanihata ◽  
H Hayashi ◽  
Y Ichikawa ◽  
...  
Keyword(s):  
Adverse Events ◽  
Ischemic Cardiomyopathy ◽  
Cox Regression ◽  
Intramuscular Fat ◽  
Left Ventricular ◽  
Skeletal Muscle Atrophy ◽  
Measured Quantity ◽  
Ectopic Fat ◽  
Cox Regression Analysis ◽  
Kaplan Meier

Abstract Background Skeletal muscle atrophy is an independent prognostic predictor for patients with chronic heart failure, and the concept of sarcopenia is drawing attention. Furthermore, the importance of not only muscle mass but also intramuscular fat (IMF) has been pointed out. However, there is a lack of consensus on the implications of ectopic fat for the prognosis in patients with non-ischemic cardiomyopathy. Purpose We investigated whether ectopic fat in the thigh affects the prognosis with non-ischemic cardiomyopathy. Methods We recruited 105 patients who were diagnosed with non-ischemic cardiomyopathy by cardiac catheterization and echocardiographic date between September 2017 and November 2019. Finally 73 patients with reduced EF (EF 40% or less) enrolled in this prospective study. Functional status was evaluated by using cardiopulmonary exercise test at baseline. All patients were measured quantity of epicardial fat and thigh IMF percentage (%IMF) using computed tomography scan. Demographic, laboratory and echocardiographic date were collected from the patients' medical records. Clinical endpoints were unexpected readmission. Results During the follow-up period 18 patients had adverse events. The %IMF was significantly higher in the group with adverse events than without (5.57±5.70 and 3.02±2.44%, respectively; p<0.01). Spearman correlation coefficient analysis showed a modest correlation between %IMF and lower limb extension strength (Spearman r=−0.280; p=0.0315), but there was no significant correlation between %IMF and exercise tolerance such as anaerobic threshold and peak oxygen uptake. Patients were divided into 2 groups according to the median values of %IMF. Kaplan-Meier analysis demonstrated that events were significantly higher in the high %IMF group (log-rank p=0.033). Multivariate Cox regression analysis adjusted for left ventricular end-diastolic diameter and peak ventricular oxygen consumption found %IMF as an independent factor of adverse events (hazard ratio 1.545; 95% confidential interval 1.151–2.087; p=0.004). Conclusions In non-ischemic cardiomyopathy patients with reduced EF, %IMF may have important adverse consequences such as increased cardiac-related events. Kaplan-Meier curves Funding Acknowledgement Type of funding source: None

scholarly journals Implications of multiple late gadolinium enhancement lesions on the frequency of left ventricular reverse remodeling and prognosis in patients with non‐ischemic cardiomyopathy

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Shingo Ota ◽  
Makoto Orii ◽  
Tsuyoshi Nishiguchi ◽  
Mao Yokoyama ◽  
Ryoko Matsushita ◽  
...  
Keyword(s):  
Heart Failure ◽  
Late Gadolinium Enhancement ◽  
Ischemic Cardiomyopathy ◽  
Left Ventricular ◽  
Reverse Remodeling ◽  
Cardiac Events ◽  
Gadolinium Enhancement ◽  
Systolic Volume ◽  
Linear Pattern ◽  
Multiple Lesions

Abstract Background Non-ischemic cardiomyopathy (NICM) is a heterogeneous disease, and its prognosis varies. Although late gadolinium enhancement (LGE)-cardiovascular magnetic resonance (CMR) demonstrates a linear pattern in the mid-wall of the septum or multiple LGE lesions in patients with NICM, the therapeutic response and prognosis of multiple LGE lesions have not been elucidated. This study aimed to investigate the frequency of left ventricular (LV) reverse remodeling (LVRR) and prognosis in patients with NICM who have multiple LGE lesions. Methods This single-center retrospective study included 101 consecutive patients with NICM who were divided into 3 groups according to LGE-CMR results: patients without LGE (no LGE group = 48 patients), patients with a typical mid-wall LGE pattern (n = 29 patients), and patients with multiple LGE lesions (n = 24 patients). LVRR was defined as an increase in LV ejection fraction (LVEF) ≥ 10 % and a final value of LVEF > 35 %, which was accompanied by a decrease in LV end-systolic volume ≥ 15 % at 12-month follow-up using echocardiography. The frequency of composite cardiac events, defined as sudden cardiac death (SCD), aborted SCD (non-fatal ventricular fibrillation, sustained ventricular tachycardia, or adequate implantable cardioverter-defibrillator therapies), and heart failure death or hospitalization for worsening heart failure, were summarized and compared between the groups. Results Among the 3 groups, the frequency of LVRR was significantly lower in the multiple lesions group than in the no LGE and mid-wall groups (no LGE vs. mid-wall vs. multiple lesions: 49 % vs. 52 % vs. 19 %, p = 0.03). There were 24 composite cardiac events among the patients: 2 in patients without LGE (hospitalization for worsening heart failure; 2), 7 in patients of the mid-wall group (SCD; 1, aborted SCD; 1 and hospitalization for worsening heart failure; 5), and 15 in patients of the multiple lesions group (SCD; 1, aborted SCD; 8 and hospitalization for worsening heart failure; 6). The multiple LGE lesions was an independent predictor of composite cardiac events (hazard ratio: 11.40 [95 % confidence intervals: 1.49−92.01], p = 0.020). Conclusions Patients with multiple LGE lesions have a higher risk of cardiac events and poorer LVRR. The LGE pattern may be useful for an improved risk stratification in patients with NICM.

scholarly journals A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation

2021 ◽  
Vol 50 (1) ◽  
Author(s):  
Josee Paradis ◽  
Agnieszka Dzioba ◽  
Hamdy El-Hakim ◽  
Paul Hong ◽  
Frederick K. Kozak ◽  
...  
Keyword(s):  
Respiratory Distress ◽  
Clinical Presentation ◽  
Tertiary Care ◽  
Choanal Atresia ◽  
Case Series ◽  
Retrospective Chart Review ◽  
National Study ◽  
Feeding Difficulties ◽  
Presenting Symptoms ◽  
Tertiary Care Centers

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract

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