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Otto Daniels

Publications and source records attributed to Otto Daniels.

7 recordsLinked to original sources
PubMed2006

Right ventricular function in asymptomatic individuals with a systemic right ventricle.

BACKGROUND: In congenital heart defects where the morphologic right ventricle (RV) supports the systemic circulation, RV failure is common yet develops gradually. We hypothesized that patients who are asymptomatic may have unrecognized RV dysfunction. METHODS: Conventional and Doppler tissue/strain echocardiography were performed on consecutive patients with asymptomatic systemic RV caused by congenitally corrected transposition of the great arteries (ccTGA) and on age-matched control subjects. RV index of myocardial performance was measured using conventional echocardiography. Longitudinal tissue velocities, strain rate, and strain of the basal RV free wall were measured using Doppler tissue/strain echocardiography and compared with nonsystemic RV of the control subjects. RESULTS: Mean age was 39.5 +/- 14.6 (n = 13) and 36 +/- 24 (n = 10) years for ccTGA and control groups, respectively. Mean RV index of myocardial performance was higher in patients with ccTGA than in control subjects (0.66 +/- 0.25 vs 0.28 +/- 0.12, P < .001). Mean RV tissue displacement (10.8 +/- 4.5 vs 20.3 +/- 3.9 mm, P < .0001), peak systolic strain rate (-1.16 +/- 0.3 vs -2.23 +/- 0.9 s(-1), P = .005), and peak systolic strain (-17.3 +/- 8.0 vs -30.6 +/- 11.0%, P = .008) were significantly lower in patients with ccTGA compared with control subjects, respectively. CONCLUSION: Patients with asymptomatic ccTGA have quantifiable RV dysfunction by echocardiography.

Adult↗
PubMed2006

MTRR 66A>G polymorphism in relation to congenital heart defects.

BACKGROUND: Evidence is accumulating that periconceptional folic acid supplementation may prevent congenital heart defects (CHD). The methionine synthase reductase (MTRR) enzyme restores methionine synthase (MTR) enzyme activity and therefore plays an essential role in the folate- and vitamin B(12)-dependent remethylation of homocysteine to methionine. We studied the influence of the MTRR 66A>G polymorphism on CHD risk. In addition, possible interaction between this variant and plasma methylmalonic acid (MMA) concentrations, as an indicator of intracellular vitamin B(12) status, was investigated. METHODS: Case-control and case-parental studies were conducted to explore this association. In total, 169 CHD patients and 213 child controls, and 159 mothers with a CHD-affected child and 245 female controls were included. RESULTS: The maternal MTRR 66AG and GG vs. AA genotypes revealed an odds ratio (OR) of 1.3 (95% CI 0.72-2.20) and 1.3 (0.71-2.37), respectively. Family-based transmission disequilibrium analysis did not reveal a significant association of the foetal 66G allele with the development of a heart defect in children (chi(2)=2.94, p=0.086). Maternal 66GG genotype in combination with high MMA concentration (above the 80th percentile) was associated with a three-fold (OR 3.3, 95% CI 0.86-12.50) increased risk for all types of CHD in offspring. CONCLUSIONS: These data indicate that maternal MTRR 66A>G polymorphism is not a risk factor for CHD. Maternal MTRR 66GG genotype with compromised vitamin B(12) status may possibly result in increased CHD risk. In addition to folate, vitamin B(12) supplementation may contribute to the prevention of CHD.

Adolescent↗
PubMed2004

Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries.

OBJECTIVES: The goal of this research was to identify predictors for sudden death (SD) in patients with transposition of the great arteries (TGA) who have undergone atrial inflow repair. BACKGROUND: Sudden death is the most common cause of late death after atrial inflow repair of TGA. Little is known about the predictors of SD. METHODS: This was a retrospective, multicenter, case-controlled study. We identified 47 patients after Mustard's or Senning's operation who experienced an SD event (34 SD, 13 near-miss SD). Each patient was matched with two controls with the same operation, but without an SD event. Information on numerous variables before the event was obtained and compared with controls at the same time frame. RESULTS: Presence of symptoms of arrhythmia or heart failure at most recent follow-up and history of documented arrhythmia (atrial flutter [AFL]/atrial fibrillation [AF]) were found to increase the risk of SD. Electrocardiogram (ECG), chest X-ray, and Holter ECG findings were not predictive of SD. Neither medication nor pacing was found to be protective. Most SD events (81%) occurred during exercise. Ventricular tachycardia/ventricular fibrillation were the recorded rhythm during SD in 21 of 47 patients. CONCLUSIONS: Presence of symptoms and documented AFL/AF are the best predictors of SD in TGA patients. Patients with these findings should be further evaluated for risk of SD.

Arrhythmias, Cardiac↗
PubMed2004

Measurement of the ductal L-R shunt during extracorporeal membrane oxygenation in the lamb.

OBJECTIVE: In neonates, initially a ductal shunt is often observed during veno-arterial extracorporeal membrane oxygenation (ECMO). Depending on the degree of pulmonary hypertension in these patients, the ductal shunt will be right to left (R-L), left to right (L-R), or bidirectional. A ductal L-R shunt will possibly lead to pulmonary hyperperfusion and interact with ECMO weaning. The aim of this study was to give more insight in this ductal L-R shunt during ECMO by quantification of this shunt in relation to cardiac output and ECMO flow. METHODS: In 7 lambs, closure of the duct was prevented by infiltration of the ductal wall with 10% formaline. This patent duct could be closed using a vesselloop around the duct. Ultrasound flowprobes were installed around the pulmonary artery, ascending aorta, and around the ECMO circulation tube. Right and left ventricular output and ECMO flow were measured. Ductus flow was defined as ductal left to right shunt (Qduct L-R) = flow in ascending aorta (Qao) - flow in central pulmonary artery (Qpa) and Qduct R-L = Qpa = Qao. RESULTS: In 6 of 7 lambs a ductal L-R shunt was observed with a mean shunt of 44% (range, 11 to 79) of left ventricular output (Qduct L-R/Qao). Comparison with ECMO flow (Qduct L-R/Qecmo) showed a mean shunt of 76% (range 15 to 230). When compared with the total systemic circulating volume (Qpa + flow in the ECMO circuit [Qecmo]), the mean ductal L-R shunt showed a percentage of 51% (range, 7% to 142%). CONCLUSIONS: During ECMO, mostly a ductal L-R shunt is observed in this lamb model. This ductal shunt is hemodynamically important. The percentages of this shunt in comparison with left ventricular output, and total circulating volume will support the idea that a ductal L-R shunt during ECMO could be another deteriorating factor in the often critical circulation of the neonate on veno-arterial ECMO.

Animals↗
PubMed2002

Connexin 43 (GJA1) mutations cause the pleiotropic phenotype of oculodentodigital dysplasia.

Gap junctions are assemblies of intercellular channels that regulate a variety of physiologic and developmental processes through the exchange of small ions and signaling molecules. These channels consist of connexin family proteins that allow for diversity of channel composition and conductance properties. The human connexin 43 gene, or GJA1, is located at human chromosome 6q22-q23 within the candidate region for the oculodentodigital dysplasia locus. This autosomal dominant syndrome presents with craniofacial (ocular, nasal, and dental) and limb dysmorphisms, spastic paraplegia, and neurodegeneration. Syndactyly type III and conductive deafness can occur in some cases, and cardiac abnormalities are observed in rare instances. We found mutations in the GJA1 gene in all 17 families with oculodentodigital dysplasia that we screened. Sixteen different missense mutations and one codon duplication were detected. These mutations may cause misassembly of channels or alter channel conduction properties. Expression patterns and phenotypic features of gja1 animal mutants, reported elsewhere, are compatible with the pleiotropic clinical presentation of oculodentodigital dysplasia.

Adolescent↗