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Outcomes of Cardiac Screening in Adolescent Soccer Players Malhotra, Aneil; Dhutia, Harshil; Finocchiaro, Gherardo ...
New England journal of medicine/The New England journal of medicine,
08/2018, Volume:
379, Issue:
6
Journal Article
Peer reviewed
Open access
A cohort of 11,168 adolescent soccer players in the United Kingdom underwent cardiac screening. Diseases associated with sudden cardiac death were identified in 42 (0.38%). The incidence of sudden ...cardiac death was 1 per 14,800 person-years, or 6.8 per 100,000 athletes.
The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a ...growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.
Aortic diseases may be diagnosed after a long period of subclinical development or they may have an acute presentation. Acute aortic syndrome is often the first sign of the disease, which needs rapid ...diagnosis and decision making to reduce the extremely poor prognosis. Aortic dilatation is a well-recognised risk factor for acute events and can occur as a result of trauma, infection, or, most commonly, from an intrinsic abnormality in the elastin and collagen components of the aortic wall. Over the years it has become clear that a few monogenic syndromes are strongly associated with aneurysms and often dictate a severe presentation in younger patients while the vast majority have a multifactorial pathogenesis. Conventional cardiovascular risk factors and ageing play an important role. Management strategy is based on prevention consisting of regular follow-up with cross-sectional imaging, chemoprophylaxis of further dilatation with drugs proved to slow down the disease progression and preventative surgery when dimension exceeds internationally recognised cut-off values for aortic diameters and the risk of rupture/dissection is therefore deemed very high.
The Fontan procedure provides palliation for patients with complex congenital heart disease. A late complication is the development of a pro-arrhythmogenic environment. Modifications to the surgical ...technique try and reduce the incidence of late arrhythmia. This study aims to establish incidence and outcome of arrhythmias in Fontan patients with long-term follow-up.
Retrospective analysis of adult patients with a Fontan circulation cared for by University Hospitals Birmingham between 2001 and 2013.
166 patients, mean age 29.1 years, were identified (63% atriopulmonary (AP) Fontan, 13% lateral tunnel (LT), 24% extracardiac total cavopulmonary connection (TCPC)). The mean follow-up since Fontan surgery was 18.6 years. 42% (70 patients) had suffered at least one tachyarrhythmia, with 100% incidence of arrhythmia in AP Fontan patients surviving at least 26 years following surgery. The most common arrhythmia was intra-atrial re-entrant tachycardia (66%). There was also a significant incidence of arrhythmia in the LT and TCPC groups (23% at mean follow-up of 19.6 years and 13.6 years, respectively). 44 patients had undergone electrical cardioversion, 30 had ablations and 10 had undergone Fontan conversion surgery. Survival analysis showed only age at the time of Fontan as a significant predictor for arrhythmia onset (p<0.001) irrespective of surgical approach.
There is a significant, increasing arrhythmia burden in adult patients with a Fontan circulation. Arrhythmia development could be regarded as an inevitable consequence of an AP Fontan. There remains a high incidence of arrhythmia with more modern surgical approaches. The long-term future of these patients is unclear and their care poses significant challenges.
Long-term fate of the aortic valve after an arterial switch operation Lo Rito, Mauro, MD; Fittipaldi, Mario, MD; Haththotuwa, Randula, MD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
04/2015, Volume:
149, Issue:
4
Journal Article
Peer reviewed
Open access
Abstract Objective To evaluate long-term performance of the aortic valve after an arterial switch operation (ASO), in terms of regurgitation and reoperation. Methods A longitudinal analysis of ...patients who underwent an ASO between 1988 and 1998 at the Birmingham Children's Hospital. Any type of aortic valve surgery after ASO was censored. To determine progression of aortic valve regurgitation, cardiology follow-up and echocardiograms from the operation until 2012 were reviewed. Results A total of 362 patients underwent ASO; median age was 8 days. Ventricular septal defect was present in 151, aortic coarctation in 39, and interrupted aortic arch in 6 patients. The trap door technique was used for coronary translocation when possible; 4.1% had intramural coronaries. There were 331 alive patients; follow-up was complete at 93%; median duration was 16 years (interquartile range, 12-18.2 years). Overall survival at 10 and 20 years was 97.6% and 95.2%, respectively. Aortic valve reoperation occurred in 5 patients, giving freedom from aortic surgery of 99.3% and 97.7% at 10 and 20 years, respectively. Reoperations were aortic valve replacement (3), Bentall operation (1), and aortic valve repair (1). Freedom from ≥moderate regurgitation at 10 with 20 years was 97% to 80%, respectively. Degree of regurgitation immediately after ASO was strongly predictive of late aortic valve function: patients discharged with mild regurgitation had a significantly increased risk of progression compared with those with no regurgitation (20 years of freedom from ≥moderate regurgitation, 50% vs 96%, P = .0000). Conclusions Reoperation on the aortic valve is rarely necessary, even late after an ASO, but a significant number of patients do have late aortic regurgitation and continue to need observation.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
To evaluate time trends in the use of catheter and surgical procedures, and associated survival in isolated congenital shunt lesions.
Nationwide, retrospective observational study of the UK National ...Congenital Heart Disease Audit database from 2000 to 2016. Patients undergoing surgical or catheter procedures for atrial septal defect (including sinus venosus defect), patent foramen ovale, ventricular septal defect and patent arterial duct were included. Temporal changes in the frequency of procedures, and survival at 30 days and 1 year were determined.
40 911 procedures were performed, 16 604 surgical operations and 24 307 catheter-based interventions. Transcatheter procedures increased over time, overtaking surgical repair in 2003-2004, while the number of operations remained stable. Trends in interventions differed according to defect type and patient age. Catheter closure of atrial septal defects is now more common in children and adults, although surgical interventions have also increased. Patent foramen ovale closure in adults peaked in 2009-2010 before falling significantly since. Surgery remains the mainstay for ventricular septal defect in infants and children. Duct ligation is most common in neonates and infants, while transcatheter intervention is predominant in older children. Excluding duct ligation, survival following surgery was 99.4% and ≈98.7%, and following catheter interventions was 99.7% and ≈99.2%, at 30 days and 1 year, respectively.
Trends in catheter and surgical techniques for isolated congenital shunt lesions plot the evolution of the specialty over the last 16 years, reflecting changes in clinical guidelines, technology, expertise and reimbursement, with distinct patterns according to lesion and patient age.
Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The ...purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation.
A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described.
Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1–50) months.
HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.
•HCC is asymptomatic at the time of diagnosis in 50% of Fontan patients.•26% of Fontan patients with HCC have a normal alpha-fetoprotein.•1-year survival after HCC diagnosis in patients with a Fontan circulation is 50%.•Meticulous liver surveillance is important in patients with a Fontan circulation.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
In patients with an extra-cardiac Fontan circulation, there is no direct access to the heart. The insertion of a permanent pacemaker requires surgery to insert epicardial pacing wires. We present the ...implantation of a permanent endocardial pacing lead from the superior vena cava (SVC) into the atrium via direct passage from the right pulmonary artery (RPA).
A permanent pacing lead was passed directly from the SVC to the RPA and then into the atrial mass. Direct passage from the RPA (attached directly to the right SVC) into the atrial mass was achieved using a trans-septal puncture needle.
This novel technique is an alternative to epicardial pacing in patients with an extra-cardiac Fontan circulation, thus avoiding the need for surgical intervention. It may also be applied to gain access to the atrial mass for arrhythmia ablation therapy.
Abstract Background Pregnancy is associated with an increased risk of aortic pathology. We sought to assess the feasibility of performing non-contrast 3D steady-state free-precession (SSFP) magnetic ...resonance angiography (MRA) in pregnant subjects with inherited aortopathy. Methods Fifteen pregnant subjects (age 27 ± 4 yr) with positive genotyping for aortopathy (Marfan, Loeys-Dietz, Ehlers-Danlos) and/or a family history of aortic dissection underwent non-contrast 3D–SSFP MRA at 1.5 T (Avanto, Siemens Healthcare, Erlangen, Germany) using a modified ECG-triggered orientated in a sagittal-oblique plane with a respiratory navigator at the diaphragmatic level (mean acquisition time 4.1 ± 1.9 min). Imaging was performed during the mid-trimester (21 ± 5 weeks). Image analysis was performed off-line using Cvi42 software (Circle Cardiovascular Imaging, Calgary, Canada). An assessment of image quality (score 0–3) was made before performing inner edge to inner edge measurements of the thoracic aorta at 7 levels from the multiplanar reconstructions by two independent blinded observers. Results Non-contrast 3D–MRA was successfully acquired in all 15 subjects. Image quality was deemed excellent in 87% (13/15) of cases after a mean acquisition time of 4.1 ± 1.9 min. There was a high level of agreement for aortic measurements, with low intra- and inter-observer variability (ICC ranges; 0.95–0.99 and 0.92–0.98, respectively). All pregnancies reached term (≥ 37/40) with a mean gestation at delivery of 38.0 ± 0.5 weeks. The mode of delivery was vaginal in 9 out of 15 subjects (60%). Conclusions Non-contrast SSFP MRA imaging provides a quick and reproducible method of assessing the thoracic aorta in pregnancy.
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