scholarly journals Fontan circulation and implications for future reproduction

2016 ◽  
Vol 10 (3) ◽  
pp. 104-106
Author(s):  
Niharika Mehta ◽  
Srilakshmi Mitta
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Cardiac Complications ◽  
Preconception Counseling ◽  
Passive Flow ◽  
History Of ◽  
Future Reproduction ◽  
Long Term Prognosis

This article summarizes and critiques four recent publications looking at preconception counseling, pregnancy outcomes, and cardiac complications in women with history of Fontan circulation. The Fontan procedure is a palliative strategy for single-ventricle type congenital heart disease and involves passive flow of venous return into the pulmonary circulation, bypassing the ventricles. Pregnancy in these patients is not without risk and preconception counseling and contraception practices vary widely. High rates of miscarriage, prematurity, and small-for-gestational-age babies are reported. Cardiac complications include mainly arrhythmias. Whether long-term prognosis in these patients is affected by pregnancy is not yet known.

Developing an adolescent and adult Fontan Management Programme

2021 ◽  
pp. 1-6
Author(s):  
Adam M. Lubert ◽  
Tarek Alsaied ◽  
Andrew T. Trout ◽  
Jonathan R. Dillman ◽  
Joseph J. Palermo ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Multiple Organ ◽  
Fontan Circulation ◽  
Management Programme ◽  
Adverse Outcomes ◽  
Cardiac Complications ◽  
Organ Systems ◽  
Adolescents And Adults ◽  
Care Models

Abstract Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

scholarly journals LONG TERM OUTCOMES AFTER THE FONTAN PROCEDURE IN PATIENTS WITH SINGLE VENTRICLE: A META-ANALYSIS

2017 ◽  
Vol 69 (11) ◽  
pp. 622
Author(s):  
Ilana Schwartz ◽  
Courtney McCracken ◽  
Christopher Petit ◽  
Ritu Sachdeva
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Fontan Procedure ◽  
Long Term Outcomes

Adenoid Cystic Carcinoma With Sialolithiasis of the Left Submandibular Gland: A Case Report and Literature Review

2018 ◽  
Vol 27 (3) ◽  
pp. 305-310 ◽  
Author(s):  
Satoru Miyabe ◽  
Kenichiro Ishibashi ◽  
Kosuke Saida ◽  
Yukio Fujiyoshi ◽  
Hideo Fukano ◽  
...  
Keyword(s):  
Adenoid Cystic Carcinoma ◽  
Submandibular Gland ◽  
Surgical Specimen ◽  
Adenoid Cystic ◽  
History Of ◽  
Supraomohyoid Neck Dissection ◽  
Additional Procedure ◽  
Long Term Prognosis ◽  
Attending Physician

Adenoid cystic carcinoma is one of the most common salivary gland malignancies with poor long-term prognosis, but the coexistence of sialoliths is extraordinarily rare. In this article, we report a case of 30-year-old woman with a history of submandibular area swelling with intermittent pain increasing during mealtimes that had led her attending physician to diagnose a sialolith in the left submandibular gland on a radiograph 10 years before. However, the surgical specimen proved to be an adenoid cystic carcinoma accompanied with a sialolith. Histopathologically, the submandibular gland was displaced with a fibrous granulation tissue containing a small cribriform carcinoma invading the extracapsular region of the gland. We performed fluorescence in situ hybridization examination with an MYB-NFIB fusion probe of the lesion, with positive results. The patient underwent a supraomohyoid neck dissection as additional procedure because of the possibility of the extracapsular cancer nest remaining around the submandibular gland, but she remains well and disease free 11 years after the first operation.

scholarly journals Neuropsychological sequelae in Kleine-Levin syndrome: case report

2000 ◽  
Vol 58 (2B) ◽  
pp. 531-534 ◽  
Author(s):  
LEONARDO FONTENELLE ◽  
MAURO V. MENDLOWICZ ◽  
J. CHRISTIAN GILLIN ◽  
PAULO MATTOS ◽  
MÁRCIO VERSIANI
Keyword(s):  
Case Report ◽  
Early Intervention ◽  
Natural History ◽  
Typical Case ◽  
Social Functions ◽  
Behavioral Disturbances ◽  
Neuropsychological Sequelae ◽  
History Of ◽  
Long Term Prognosis

Kleine-Levin syndrome is characterized by periodic hypersomnia, hyperphagia, sexual disinhibitions and behavioral disturbances. The prognosis is generally benign, with normal cognitive and social functions after the episodes. We describe a typical case of Kleine-Levin syndrome associated with apparent academic decline, neuropsychological sequelae and personality alterations after the second episode of the illness. Further research in the natural history of Kleine-Levin syndrome is needed, for example, to determine whether early intervention would improve long-term prognosis.

scholarly journals Beyond Sector Retinitis Pigmentosa: Expanding the Phenotype and Natural History of the Rhodopsin Gene Codon 106 Mutation (Gly-to-Arg) in Autosomal Dominant Retinitis Pigmentosa

Genes ◽  
2021 ◽  
Vol 12 (12) ◽  
pp. 1853
Author(s):  
Brian G. Ballios ◽  
Emily M. Place ◽  
Luis Martinez-Velazquez ◽  
Eric A. Pierce ◽  
Jason I. Comander ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Autosomal Dominant ◽  
Peripheral Vision ◽  
Visual Fields ◽  
Macular Disease ◽  
History Of ◽  
Intrafamilial Variability ◽  
Long Term Prognosis

Sector and pericentral are two rare, regional forms of retinitis pigmentosa (RP). While usually defined as stable or only very slowly progressing, the available literature to support this claim is limited. Additionally, few studies have analyzed the spectrum of disease within a particular genotype. We identified all cases (9 patients) with an autosomal dominant Rhodopsin variant previously associated with sector RP (RHO c.316G > A, p.Gly106Arg) at our institution. Clinical histories were reviewed, and testing included visual fields, multimodal imaging, and electroretinography. Patients demonstrated a broad phenotypic spectrum that spanned regional phenotypes from sector-like to pericentral RP, as well as generalized disease. We also present evidence of significant intrafamilial variability in regional phenotypes. Finally, we present the longest-reported follow-up for a patient with RHO-associated sector-like RP, showing progression from sectoral to pericentral disease over three decades. In the absence of comorbid macular disease, the long-term prognosis for central visual acuity is good. However, we found that significant progression of RHO p.Gly106Arg disease can occur over protracted periods, with impact on peripheral vision. Longitudinal widefield imaging and periodic ERG reassessment are likely to aid in monitoring disease progression.

Current treatment options for the Failing Fontan Circulation

2022 ◽  
Vol 18 ◽  
Author(s):  
Bart. W. Driesen ◽  
Michiel Voskuil ◽  
Heynric B. Grotenhuis
Keyword(s):  
Myocardial Dysfunction ◽  
Fontan Procedure ◽  
Treatment Options ◽  
Fontan Operation ◽  
Current Treatment ◽  
Fontan Circulation ◽  
Protein Losing Enteropathy ◽  
Resistance Protein ◽  
Failing Fontan

Abstract: The Fontan operation was introduced in 1968. For congenital malformations where biventricular repair is not suitable, the Fontan procedure has provided a long-term palliation strategy with improved outcome compared to the initially developed procedures. Despite these improvements, several complications merely as a result of a failing Fontan circulation (including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein losing enteropathy, hepatic dysfunction, plastic bronchitis and thrombo-embolism) will limit life-expectancy in this patient cohort. This review provides an overview of the most common complications of the Fontan circulation and the currently available treatment options.

Long-term prognosis of patients withJ-wave syndrome

Heart ◽  
2019 ◽  
Vol 106 (4) ◽  
pp. 299-306
Author(s):  
Tsukasa Kamakura ◽  
Tetsuji Shinohara ◽  
Kenji Yodogawa ◽  
Nobuyuki Murakoshi ◽  
Hiroshi Morita ◽  
...  
Keyword(s):  
High Amplitude ◽  
Long Term Study ◽  
St Segment ◽  
History Of ◽  
Provocation Testing ◽  
Long Term Prognosis ◽  
Electrocardiographic Parameters ◽  
The Impact

ObjectiveLimited data are currently available regarding the long-term prognosis of patients with J-wave syndrome (JWS). The aim of this study was to investigate the long-term prognosis of patients with JWS and identify predictors of the recurrence of ventricular fibrillation (VF).MethodsThis was a multicentre retrospective study (seven Japanese hospitals) involving 134 patients with JWS (Brugada syndrome (BrS): 85; early repolarisation syndrome (ERS): 49) treated with an implantable cardioverter defibrillator. All patients had a history of VF. All patients with ERS underwent drug provocation testing with standard and high intercostal ECG recordings to rule out BrS. The impact of global J waves (type 1 ECG or anterior J waves and inferolateral J waves in two or more leads) on the prognosis was evaluated.ResultsDuring the 91±66 months of the follow-up period, 52 (39%) patients (BrS: 37; ERS: 15) experienced recurrence of VF. Patients with BrS and ERS with global J waves showed a significantly higher incidence of VF recurrence than those without (BrS: log-rank, p=0.014; ERS: log-rank, p=0.0009). The presence of global J waves was a predictor of VF recurrence in patients with JWS (HR: 2.16, 95% CI 1.21 to 3.91, p=0.0095), while previously reported high-risk electrocardiographic parameters (high-amplitude J waves ≥0.2 mV and J waves associated with a horizontal or descending ST segment) were not predictive of VF recurrence.ConclusionsThis multicentre long-term study showed that the presence of global J waves was associated with a higher incidence of VF recurrence in patients with JWS.

scholarly journals PREVALENCE AND LONG TERM PROGNOSIS OF PATIENTS WITH COMPLETE LEFT BUNDLE BRANCH BLOCK AND WITHOUT A PRIOR HISTORY OF HEART DISEASE REFERRED FOR NUCLEAR STRESS SPECT

2012 ◽  
Vol 59 (13) ◽  
pp. E1183 ◽  
Author(s):  
Azhar Supariwala ◽  
Seth Uretsky ◽  
Sonal Kamalia ◽  
Narasimhanaidu Guriginjakunta ◽  
Madhusudhan Ponnala ◽  
...  
Keyword(s):  
Heart Disease ◽  
Left Bundle Branch Block ◽  
Prior History ◽  
Bundle Branch Block ◽  
History Of ◽  
Long Term Prognosis

Long term prognosis of the coronary artery with negative echo findings in the cases with a history of Kawasaki disease

1992 ◽  
Vol 1 (1) ◽  
pp. 78
Author(s):  
Tetsuro Kamiya ◽  
Atsuko Suzuki ◽  
Yasuo Ono ◽  
Yoshio Arakaki ◽  
Kenji Kuroe ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
History Of ◽  
Long Term Prognosis

scholarly journals Prevalence and Outcomes of Achromobacter Species Infections in Adults with Cystic Fibrosis: a North American Cohort Study

2017 ◽  
Vol 55 (7) ◽  
pp. 2074-2085 ◽  
Author(s):  
B. D. Edwards ◽  
J. Greysson-Wong ◽  
R. Somayaji ◽  
B. Waddell ◽  
F. J. Whelan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Persistent Infection ◽  
Lung Function Decline ◽  
Multicenter Studies ◽  
Content Type ◽  
History Of ◽  
Long Term Prognosis ◽  
North American Cohort

ABSTRACTAchromobacterspecies are increasingly being detected in cystic fibrosis (CF) patients, with an unclear epidemiology and impact. We studied a cohort of patients attending a Canadian adult CF clinic who had positive sputum cultures forAchromobacterspecies in the period from 1984 to 2013. Infection was categorized as transient or persistent (≥50% positive cultures for 1 year). Those with persistent infection were matched 2:1 with age-, sex-, and time-matched controls without a history ofAchromobacterinfection, and mixed-effects models were used to assess pulmonary exacerbation (PEx) frequency and lung function decline. Isolates from a biobank were retrospectively assessed, identified to the species level bynrdAsequencing, and genotyped using pulsed-field gel electrophoresis (PFGE). Thirty-four patients (11% of those in our clinic), with a median age of 24 years (interquartile range [IQR], 20.3 to 29.8 years), developedAchromobacterinfection. Ten patients (29%) developed persistent infection. Persistence did not denote permanence, as most patients ultimately cleared infection, often after years. Patients were more likely to experience PEx at incident isolation than at prior or subsequent visits (odds ratio [OR], 2.7 [95% confidence interval {CI}, 1.2 to 6.7];P= 0.03). Following persistent infection, there was no difference in annual lung function decline (−1.08% [95% CI, −2.73 to 0.57%] versus −2.74% [95% CI, −4.02 to 1.46%];P= 0.12) or the odds of PEx (OR, 1.21 [95% CI, 0.45 to 3.28];P= 0.70). Differential virulence amongAchromobacterspecies was not observed, and no cases of transmission occurred. We demonstrated that incidentAchromobacterinfection was associated with a greater risk of PEx; however, neither transient nor chronic infection was associated with a worsened long-term prognosis. Large, multicenter studies are needed to clarify the clinical impact, natural history, and transmissibility ofAchromobacter.

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