Graham Stuart

Effects of atenolol, perindopril and verapamil on haemodynamic and vascular function in Marfan syndrome - a randomised, double-blind, crossover trial

Eur J Clin Invest 2012

Single-centre use of implantable loop recorders in patients with congenital heart disease

AIMS To determine the efficacy of the implantable loop recorder (ILR) in patients with congenital heart disease. METHODS AND RESULTS Retrospective data analyses of all patients with congenital heart disease undergoing ILR implantation in a congenital cardiac centre. Of 78 ILRs implanted between July 2000 and October 2007, 22 (15 male) patients had congenital heart disease. The median age at implantation was 15.5 years (7.0-46.5). Six patients had neurodevelopmental delay. Indications for implantation were syncope (n = 15), palpitations (n = 4), and pre-syncope (n = 3). Median implantation time was 19 months (1-30). Explantation data were available in 16 patients (73%). Implantable loop recorders were explanted following establishment of positive diagnosis (n = 3), negative diagnosis (n = 7), and at the end of battery life (n = 4). One patient died with the ILR in situ. One device eroded after 2 months without further complication. Following ILR interrogation, diagnosed arrhythmias included ventricular tachycardia (n = 2) and sinus node dysfunction (n = 1). On the basis of these findings, two patients had intracardiac defibrillators and one patient had a dual-chamber pacemaker implanted. CONCLUSION Implantable loop recorders lead to a positive or negative arrhythmic diagnosis following explantation in 71% of patients with congenital heart disease. This is particularly beneficial in patients with neurodevelopmental delay where accurate history is often limited.

Ventricular tachycardia secondary to prolongation of the QT interval in a fetus with autoimmune mediated congenital complete heart block

We report a case where fetal echocardiography identified both complete heart block and ventricular tachycardia. The mother tested positive for anti-Ro antibodies. Prenatal detection of this unusual combination of arrhythmias prompted early postnatal evaluation, which revealed prolongation of the QT interval. Autoimmune mediated congenitally complete heart block associated with such prolongation of the QT interval has a poor prognosis. The child was successfully treated with beta blockers and implantation of a pacemaker.

Implantation of the new Medtronic LINQ™ loop recorder in an infant with ventricular tachycardia.

The Medtronic LINQ™ was inserted in an 11-month-old boy for close monitoring of rapid ventricular tachycardia. The device is much smaller than the conventional Medtronic loop recorder. The real advantage of the LINQ™ is that it automatically notifies Carelink if any ventricular tachycardia is seen, which is very advantageous for this particular type of patient. This device is ideal for close monitoring of asymptomatic yet potentially dangerous arrhythmias in smaller children.

Extraction of SelectSecure leads compared to conventional pacing leads in patients with congenital heart disease and congenital atrioventricular block

BACKGROUND SelectSecure™ pacing leads (Medtronic Inc) are increasingly being used in pediatric patients and adults with structural congenital heart disease. The 4Fr lead is ideal for patients who may require lifelong pacing and can be advantageous for patients with complex anatomy. OBJECTIVE The purpose of this study was to compare the extraction of SelectSecure leads with conventional (stylette-driven) pacing leads in patients with structural congenital heart disease and congenital atrioventricular block. METHODS The data on lead extractions from pediatric and adult congenital heart disease (ACHD) patients from August 2004 to July 2014 at Bristol Royal Hospital for Children and the Bristol Heart Institute were reviewed. Multivariable regression analysis was used to determine whether conventional pacing leads were associated with a more difficult extraction process. RESULTS A total of 57 patients underwent pacemaker lead extractions (22 SelectSecure, 35 conventional). No deaths occurred. Mean age at the time of extraction was 17.6 ± 10.5 years, mean weight was 47 ± 18 kg, and mean lead age was 5.6 ± 2.6 years (range 1-11 years). Complex extraction (partial extraction/femoral extraction) was more common in patients with conventional pacing leads at univariate (P < .01) and multivariate (P = .04) levels. Lead age was also a significant predictor of complex extraction (P < .01). CONCLUSION SelectSecure leads can be successfully extracted using techniques that are used for conventional pacing leads. They are less likely to be partially extracted and are less likely to require extraction using a femoral approach compared with conventional pacing leads.

Use of Radiofrequency Perforation for Lead Placement in Biventricular or Conventional Endocardial Pacing after Mustard or Senning Operations for D‐Transposition of the Great Arteries

Background: Endocardial pacemaker lead placement can be challenging after Mustard and Senning operations for transposition of the great arteries (D‐TGA), if there is atresia of the systemic venous pathways and because the coronary sinus cannot be used for cardiac resynchronization therapy. Radiofrequency (RF)‐assisted perforation techniques have been used in congenital heart disease but have not been reported for use in pacemaker implantation.

Adult congenital heart disease interventions: Recommendations from a Joint Working Group of the British Congenital Cardiac Association, British Cardiovascular Intervention Society, and the British Cardiovascular Society

In order to optimise care of the adult patients with complex congenital heart disease, there is a need to develop recommendations for interventions. This document is the work of representatives of the three relevant societies and provides recommendations for institutions and operators performing cardiac interventions in these patients.

Imaging and percutaneous occlusion of a large aneurysm of the ductus arteriosus in an infant with Loeys-Dietz syndrome.

Loeys-Dietz is a multisystem congenital syndrome that comprises craniofacial and cutaneous abnormalities as well as structural cardiac defects. One of its key pathological features is an aggressive widespread vasculopathy that can manifest as aortic or cerebral aneurysms, which is prone to dissection and rupture. We report a case of a large aneurysm of the ductus arteriosus in a patient with Loeys-Dietz syndrome, successfully occluded by interventional catheterization.

Cardiovascular Manifestations and Complications of Loeys-Dietz Syndrome: CT and MR Imaging Findings

Loeys-Dietz syndrome (LDS) is a recently described genetic connective tissue disorder with a wide spectrum of multisystem involvement. LDS is characterized by rapidly progressive aortic and peripheral arterial aneurysmal disease. LDS and the other inherited aortopathies such as Marfan syndrome have overlapping phenotypic features. However, LDS is characterized by a more aggressive vascular course; patient morbidity and mortality occur at an early age, with complications developing at relatively smaller aortic dimensions. In addition, there is more diffuse arterial involvement in LDS, with a large proportion of patients developing aneurysms of the iliac, mesenteric, and intracranial arteries. Early diagnosis and careful follow-up are essential for ensuring timely intervention in patients with arterial disease. Cross-sectional angiography has an important role in the baseline assessment, follow-up, and evaluation of acute complications of LDS, the thresholds and considerations of which differ from those of other inherited aortopathies. In this article, LDS is compared with other genetic vascular connective tissue disorders. In addition, the genetic, histopathologic, and cardiovascular manifestations of this disease process are reviewed, with a focus on computed tomographic and magnetic resonance imaging findings. Online DICOM image stacks and supplemental material are available for this article. ©RSNA, 2018.

Cardiopulmonary Exercise and Six-Minute Walk Testing

Cardiopulmonary exercise testing (CPEX) and the six-minute walk testing (6MWT) enable the detailed functional evaluation of patients with pulmonary hypertension secondary to congenital heart disease. These tests can be used for symptom evaluation, assessment of intervention and a guideline to prognosis. A good understanding of the physiology of cardiopulmonary exercise testing is a prerequisite for the optimal care of the adult with pulmonary hypertension secondary to congenital heart disease.