PubMed Health⌕ Search

Biomedical subjects

Paul M Weinberg

Publications and source records attributed to Paul M Weinberg.

17 recordsLinked to original sources
PubMed2006

Usefulness of magnetic resonance imaging for the diagnosis of right ventricular dysplasia in children.

Cardiac magnetic resonance (CMR) has been helpful in adults in the diagnosis of arrhythmogenic right ventricular dysplasia. Short of direct surgical observation or autopsy, no gold standard exists. CMR diagnostic criteria include right atrial and ventricular dilation, regional right ventricular (RV) wall motion abnormalities, outflow tract ectasia, and myocardial fatty infiltration. To determine whether adult diagnostic criteria are useful in children referred for CMR for this diagnosis, the images and records of 81 patients (aged 11.5 +/- 5.5 years) over an 8-year period were reviewed. Histories included ventricular tachycardia, palpitations, dilated right ventricle, syncope, near sudden death, or family history of RV dysplasia. Four families were studied with parents who had RV dysplasia diagnosed by surgery, explanted heart, or CMR. CMR imaging included T1-weighted imaging, cine, 1-dimensional RV myocardial tagging, and phase-encoded velocity mapping, and 2 patients underwent delayed-enhancement CMR. Only 1 of the 81 patients met 5 of the criteria. None of the others met >2 of the criteria, and only 2 patients met 1 or 2 criteria. For questionable regional wall motion abnormalities, RV myocardial tagging was helpful. In conclusion, CMR of patients with a history suspicious for the diagnosis of RV dysplasia is a low-yield test in children. This may be due to the evolving nature of the disease, which does not manifest itself from a morphologic or ventricular-function standpoint until later in development. Follow-up studies as patients age may be advantageous.

Cardiomyopathy, Dilated↗
PubMed2006

Heterotaxy syndrome with functional single ventricle: does prenatal diagnosis improve survival?

BACKGROUND: Despite improved outcome for many single ventricle lesions, staged reconstruction for heterotaxy syndrome with a functional single ventricle continues to have a high mortality. Prenatal identification of heterotaxy syndrome may improve long-term survival. METHODS: Our database was reviewed from January 1996 to December 2004 for patients with heterotaxy syndrome. Assessment was made for prenatal diagnosis and echocardiographic characteristics of heterotaxy syndrome. We sought to assess the accuracy of fetal echocardiography in the diagnosis of heterotaxy syndrome and determine whether prenatal diagnosis and other risk factors have an impact on survival in patients with heterotaxy syndrome. RESULTS: Of 81 patients that met criteria, 43 (53%) had prenatal diagnosis. Prenatal diagnosis had high specificity and positive predictive value for all findings but had low sensitivity for anomalous pulmonary veins. Among the 70 patients born alive, survival was 60% with median follow-up of 51.4 months (range, 6.5 to 109.7 months). Prenatal diagnosis did not improve survival (p = 0.09). None of the 11 patients with complete heart block (CHB) survived past 3 months of age. Two patients underwent heart transplantation as their first intervention and have survived. CHB and anomalous pulmonary venous connection were associated with shorter duration of survival. CONCLUSIONS: Prenatal diagnosis of heterotaxy syndrome does not improve survival in patients who undergo single ventricle reconstruction. The most potent risk factors for poor outcome (CHB, anomalous pulmonary veins) are likely not impacted by identification in utero. In light of the poor outcome, cardiac transplantation as an initial therapy may be a viable option for some patients.

Echocardiography↗
PubMed2006

The nomenclature, definition and classification of hypoplastic left heart syndrome.

The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair. The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.

Diagnosis, Differential↗
PubMed2006

Aortic arch anomalies.

Magnetic resonance imaging has become the gold standard for imaging the aortic arch with the ability to demonstrate not only the arterial branching pattern, but also the relationship of aorta and its branches to the trachea and bronchi. Examples of four major aortic arch types--left aortic arch, right aortic arch, double aortic arch and persistent fifth aortic--with most of their variations are demonstrated.

Aorta, Thoracic↗
PubMed2005

Quantifying regional right ventricular function in tetralogy of Fallot.

Right ventricular (RV) function is notoriously difficult to quantify. Patients with tetralogy of Fallot (TOF) have decreased systolic performance. We measure regional RV performance using MRI with 1-dimensional myocardial tissue tagging. By tagging cine-MRI in two views, we measured regional shortening in 12 regions throughout the RV. We image 32 pediatric patients: 21 normal patients and 11 patients with repaired TOF. We establish a normal range for each RV region. TOF patients have decreased shortening on a region-by-region basis. We conclude that regional RV performance can be measured using this technique, and that decreased performance can be demonstrated in TOF patients.

Adolescent↗
PubMed2003

Flow volume asymmetry in the right aortic arch in children with magnetic resonance phase encoded velocity mapping.

BACKGROUND: The right aortic arch is not uncommon in pediatrics. Flow dynamics in this type of aortic arch, which is important for cardiac energetics, organ perfusion, and Doppler flow calculations, have not been defined. Although there are complex secondary flow patterns, bulk axial flow makes up most of the energy use. METHODS: We examined 14 children with a right aortic arch by using through-plane phase-encoded magnetic resonance velocity mapping in the ascending and descending aorta to determine flow volume symmetry and velocity. The aortic cross section was divided into 4 quadrants aligned along the long axis of the aorta. Significance was defined as a P value <.05. RESULTS: In the ascending aorta, the posterior right quadrant demonstrated significantly greater blood flow than the other quadrants across the entire cardiac cycle (28% vs 23%-25%) and at the point of maximum flow (29% vs 22%-25%). Flow asymmetry was also present in the descending aorta; there was significantly more flow in the posterior quadrants than the anterior quadrants in total flow across the cardiac cycle (28% vs 21%-23%) and at the point of maximum flow (27%-28% vs 20%-24%). The time to maximum flow was significantly shorter in the ascending than the descending aorta (18% vs 24% of the cardiac cycle). In 10 of 14 patients, maximum velocity occurred in the right half of both the ascending and descending aorta. Flow reversal at end-systole was haphazard, occurring in all quadrants. CONCLUSION: Flow volume asymmetry exists in the ascending and descending portions of the right aortic arch, which has implications for cardiac energetics, organ perfusion, and Doppler scanning flow calculations. This information may be useful in designing improved aortic surgical reconstructions in cases of congenital heart disease.

Analysis of Variance↗
PubMed2002

Mid-term follow-up of patients with transposition of the great arteries after atrial inversion operation using two- and three-dimensional magnetic resonance imaging.

BACKGROUND: Older patients with transposition of the great arteries who have undergone an atrial inversion procedure (ATRIAL-INV) are difficult to image by echocardiography. The surgical baffles are spatially complex. OBJECTIVE: To test the hypothesis that two- and three-dimensional MRI can elucidate the spatially complex anatomy in this patient population. MATERIALS AND METHODS: Twelve patients with ATRIAL-INV, ages 16+/-4.5 years, underwent routine T1-weighted spin-echo axial imaging to obtain a full cardiac volumetric data set. Postprocessing created three-dimensional shaded surface displays and allowed for multiplanar reconstruction. Routine transthoracic echocardiography was available on all patients. RESULTS: Three-dimensional reconstruction enabled complete spatial conceptualization of the venous pathways, and allowed for precise localization of a narrowed region in the upper limb of the systemic venous pathway found in two patients. This was subsequently confirmed on angiography. Routine MRI was able to image the full extent of the venous pathways in all 12 patients. Routine transthoracic echocardiography was able to visualize proximal portions of the venous pathways in eight (67%), the distal upper limb in five (42%), and the distal lower limb in four (33%) patients, and it was able to visualize the outflow tracts in all patients. CONCLUSION: Three-dimensional reconstruction adds important spatial information, which can be especially important in stenotic regions. Routine MRI is superior to transthoracic echocardiography in delineation of the systemic and pulmonary venous pathway anatomy of ATRIAL-INV patients at mid-term follow-up. Although transesophageal echocardiography is an option, it is more invasive.

Adolescent↗
PubMed2002

Nonuniform flow dynamics in the aorta of normal children: a simplified approach to measurement using magnetic resonance velocity mapping.

PURPOSE: To determine regional flow dynamics in the normal aorta (Ao) in children. Understanding flow dynamics in children is important in cardiovascular energetics, in designing improved aortic reconstructions by crafting the surgery to mimic normal aortic flow, and in Doppler flow calculations. The objective of this study was to determine regional flow dynamics in the normal Ao in children. MATERIALS AND METHODS: We performed magnetic resonance velocity mapping on 13 subjects (ages 7.2 +/- 6.2 years) with normal Aos to determine flow dynamics in four equal quadrants in the ascending (AAo) and descending aorta (DAo) aligned along the long axis of the Ao. Statistical significance was set at P <.05. RESULTS: In the AAo, the left posterior quadrant displayed significantly less blood flow (16% +/- 5%) than the other quadrants (26-29%) over the cardiac cycle. In the DAo, both anterior quadrants carried significantly less blood flow (20% and 21%) than the posterior quadrants (27% and 32%). At maximum flow (15% +/- 5% into the cardiac cycle for the AAo; 27% +/- 15% for the DAo), there was significantly more flow in the right posterior quadrant (28% +/- 2%) than other quadrants (22-23%) in the AAo. In the DAo, both posterior quadrants had significantly higher flow rates (27% and 30%) than the anterior quadrants (21% and 22%). Maximum velocity in both the AAo and the DAo occurred in the left posterior quadrant in 10/13 at 16-24% into the cardiac cycle. At end-systole, a short flow reversal was noted in the posterior quadrants in the AAo in 11/13; in the DAo, this occurred in the anterior quadrants in 10/13. CONCLUSION: Flow dynamics in the normal Ao in children are not symmetrical; the flow distributions are asymmetric in both the AAo and the DAo throughout systole, including flow reversal related to the dicrotic notch. These results may help improve Ao surgery.

Analysis of Variance↗
PubMed2002

Coronary anatomy in congenitally corrected transposition of the great arteries.

BACKGROUND: The advent of double switch procedures for the treatment of transposition of the great arteries with L-looped ventricles, e.g. typical congenitally corrected transposition of the great arteries, has made delineation of the coronary artery anatomy in these hearts important. Previous studies have suggested a consistently inverted coronary arterial pattern. METHODS: A morphologic study was conducted of the coronary arterial anatomy of all heart specimens in our registry of approximately 2600 hearts with segmental anatomy [S,L,L] (situs solitus of the viscera and atria, ventricular L-loop, and levoposition of the aorta), with anatomical features to permit such repair. RESULTS: Twenty specimens collected between 1965 and 1993 ranged in age from 1 day to 23 years. Transposition of the great arteries or double outlet right ventricle was seen in 12 (60%). One had a single coronary artery that arose from the right anterior sinus and trifurcated. Another had the anterior descending artery arise from the right ventricular coronary artery while the circumflex arose alone, directly above the intercoronary commissure. Eccentric ostia were seen in another four, with one left ventricular coronary artery originating directly above the intercoronary commissure. Right ventricular aorta with pulmonary atresia was in eight of the total (40%). Three had eccentric ostia: one with both arteries from the left posterior sinus, and one with the left ventricular coronary over the intercoronary commissure. None of these specimens had additional coronary anomalies that would further complicate surgery. CONCLUSION: Nearly half of the specimens (45%) had coronary artery abnormalities that could have complicated, but not necessarily precluded, anatomic surgical repair.

Adolescent↗