Showing papers by "Nico A. Blom published in 2011"

Comparison of Transplacental Treatment of Fetal Supraventricular Tachyarrhythmias With Digoxin, Flecainide, and Sotalol Results of a Nonrandomized Multicenter Study

Abstract: Background—Fetal tachyarrhythmia may result in low cardiac output and death. Consequently, antiarrhythmic treatment is offered in most affected pregnancies. We compared 3 drugs commonly used to control supraventricular tachycardia (SVT) and atrial flutter (AF). Methods and Results—We reviewed 159 consecutive referrals with fetal SVT (n=114) and AF (n=45). Of these, 75 fetuses with SVT and 36 with AF were treated nonrandomly with transplacental flecainide (n=35), sotalol (n=52), or digoxin (n=24) as a first-line agent. Prenatal treatment failure was associated with an incessant versus intermittent arrhythmia pattern (n=85; hazard ratio [HR]=3.1; P<0.001) and, for SVT, with fetal hydrops (n=28; HR=1.8; P=0.04). Atrial flutter had a lower rate of conversion to sinus rhythm before delivery than SVT (HR=2.0; P=0.005). Cardioversion at 5 and 10 days occurred in 50% and 63% of treated SVT cases, respectively, but in only 25% and 41% of treated AF cases. Sotalol was associated with higher rates of prenatal AF ter...

Fever-Induced Life-Threatening Arrhythmias in Children Harboring an SCN5A Mutation

Abstract: Cardiac channelopathies caused by SCN5A mutation are well tolerated by most patients. However, the dramatic presentation of a previously healthy 4-month-old girl with life-threatening arrhythmias and the subsequent findings in the child and her family provide evidence that loss-of-function sodium channel mutations can present very early in life. An SCN5A mutation was detected in the infant, her brother, and their father. Both the siblings manifested recurrent serious arrhythmias during febrile episodes, which followed immunization, as well as fever of nonspecific origin. Management consisted of prompt antipyretic measures, hospitalization with vigorous monitoring during immunization and febrile episodes, and prevention of tachycardia-induced conduction disturbance with β-blockers.

Relationship between temporal sequence of right ventricular deformation and right ventricular performance in patients with corrected tetralogy of Fallot

Abstract: Objective Right ventricular (RV) dysfunction is common in patients with corrected tetralogy of Fallot (cToF). Abnormalities in the temporal pattern of RV mechanical activation have been observed in patients with cToF, but the relationship with RV performance remains unclear. This study characterises RV performance and the temporal sequence of RV deformation in patients with cToF and healthy controls. Study design 37 patients with cToF were compared with 18 controls. Using two-dimensional speckle tracking analysis, global RV strain was assessed. In addition, time to peak strain and the time difference between RV inlet and RV outlet (RV time delay) was assessed. Main outcome measure The relation between RV performance and RV time delay was assessed with linear regression analysis. Results RV strain was reduced in patients compared with controls (−20.9±4.3% vs −30.7±3.4%, p Conclusion In patients with cToF, RV outlet deformation is delayed, causing a reduction in RV time delay which is significantly related to impairment in RV performance.

Persistent Fetal Sinus Bradycardia Associated with Maternal Anti-SSA/Ro and Anti-SSB/La Antibodies

Abstract: Objective. To study the clinical course and outcome of fetal sinus bradycardia (SB) due to maternal antibody-induced sinus node dysfunction. Methods. We reviewed the maternal, prenatal, and postnatal findings of fetuses with SB associated with elevated maternal anti-SSA/Ro and anti-SSB/La antibodies. Results. Of the 6 cases diagnosed prenatally, 3 had isolated SB persisting after birth and had a good prognosis. Three fetuses with SB and severe myocardial involvement (congenital complete heart block and/or endocardial fibroelastosis) succumbed in utero in spite of treatment. Postmortem histopathology in 1 fetus showed inflammatory destruction of the sinus and atrioventricular nodes. SB was detected incidentally in a 7-year-old girl. She had intermittent heart block with progressive sinus arrest requiring permanent pacemaker. Conclusion. Fetal SB associated with maternal autoantibodies may persist in childhood, with a good prognosis in the absence of widespread cardiac involvement. (First Release Nov 15 2011; J Rheumatol 2011;38:2682-5; doi:10.3899/jrheum.110720)

Corrected tetralogy of Fallot: comparison of tissue doppler imaging and velocity-encoded MR for assessment of performance and temporal activation of right ventricle.

Abstract: Velocity-encoded MR imaging and tissue Doppler imaging can be used interchangeably for clinical assessment of myocardial velocity and timing of the right ventricle in healthy subjects and in patients with corrected tetralogy of Fallot.

Percutaneous Tricuspid Valve Implantation in a Fontan Patient With Congestive Heart Failure and Protein-Losing Enteropathy

Abstract: A 47-year-old patient visited our outpatient clinic. She was born with tricuspid atresia with normally connected great arteries without pulmonary stenosis (type Ic); a ventricular septal defect connected the pulmonary artery and hypoplastic right ventricle (RV) with the left ventricle. When she was 14 years old, a Fontan-Bjork procedure was performed.1 The atrial and the ventricular septal defects were closed and a valved conduit (Hancock prosthesis) was placed between the right atrium (RA) and the hypoplastic RV, thus providing pulsatile flow to the pulmonary arteries. Ten years later, the conduit was severely obstructed and replaced by a 23-mm aorta homograft. At the age of 38 years, she had atrioventricular block for which a dual-chamber pacemaker system was implanted with placement of the ventricular lead through the homograft in the RV apex. In the last 2 years, she had progressive heart failure and diarrhea. She was clinically diagnosed with protein-losing enteropathy (PLE), based on hypalbuminemia, edema, and diarrhea. Other …

Tissue-velocity magnetic resonance imaging and tissue Doppler imaging to assess regional myocardial diastolic velocities at the right ventricle in corrected pediatric Tetralogy of Fallot patients.

Abstract: OBJECTIVES In patients with corrected Tetralogy of Fallot (cToF), pulmonary regurgitation and subsequent increased right ventricular (RV) end-diastolic volume are diastolic parameters related to adverse outcome. In addition, abnormalities of the RV outflow tract (RVOT) independently promote RV dilatation in cToF patients. Tissue Doppler imaging (TDI) and tissue-velocity magnetic resonance imaging (TV-MRI) enable quantitative assessment of regional diastolic performance by measuring myocardial velocities. Assessment of regional diastolic velocities of the RV may provide insight into the relation between RVOT dysfunction and RV dilatation in cToF patients. The aim of the study was to perform a direct comparison of TV-MRI against TDI to assess regional RV diastolic velocities in cToF patients and control subjects. In addition, the relationship between regional RV diastolic velocities and RV dilatation was investigated. MATERIALS AND METHODS Thirty-four cToF patients (8-18 years) and 19 controls were studied. Early (E') and late (A') peak diastolic velocities and E'/A' ratio were assessed with TDI and TV-MRI at the RV free wall and at the RVOT. RV volumes and pulmonary regurgitation were quantified with planimetric and 3-dimensional flow MRI, respectively. RESULTS Good correlation and agreement were observed between TDI and TV-MRI at both regions of the RV (RV free wall: E': r = 0.92, mean bias: 0.5 cm/s, A': r = 0.92, mean bias: 0.4 cm/s; RVOT: E': r = 0.92, mean bias: -0.3 cm/s, A': r = 0.95, mean bias: 0.03 cm/s). With both imaging techniques, regional RV diastolic velocities were significantly reduced in cToF patients. The E'/A' ratio at the RVOT (assessed with both TDI and TV-MRI) was related to RV end-diastolic volume, even after correction for pulmonary regurgitation (TDI: P < 0.01, TV-MRI: P = 0.05). CONCLUSIONS TDI and TV-MRI can be used interchangeably for the assessment of regional diastolic velocities and performance of the RV in cToF patients and in healthy controls. Regional diastolic velocities at the RVOT are reduced in cToF patients as compared with controls. In addition to pulmonary regurgitation, impaired diastolic performance at the RVOT is independently related to RV dilatation.

Dilatation of the great arteries in an infant with marfan syndrome and ventricular septal defect.

Abstract: We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD), and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS) and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits. Eventually the diagnosis of MFS was confirmed by DNA analysis.

Brief communication - Congenital Neo-coarctation after the arterial switch operation

Abstract: Neo-coarctation following arterial switch operation (ASO) for transposition of the great arteries (TGA) is a complication that is not regularly described, but may occur. We describe five patients who developed a neo-coarctation after operation. They were diagnosed with TGA, either with or without ventricular septal defect without signs or symptoms of a coarctation. Except for one patient, all patients were reoperated for a neo-coarctation within one year after the ASO. Several explanations are discussed as a possible cause for this phenomenon.  2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.