The cerebroplacental Doppler ratio predicts postnatal outcome in fetuses with congenital heart block.

OBJECTIVE: Autoimmune-mediated congenital complete heart block (CCHB) is associated with significant perinatal morbidity and mortality, and prenatal monitoring of these fetuses is a significant challenge. The cerebroplacental Doppler ratio (CPR), defined as the ratio of the middle cerebral artery pulsatility index (MCA-PI) to the umbilical artery pulsatility index (UA-PI), is predictive of perinatal outcome in growth-restricted fetuses. This study tests the hypothesis that the CPR would be useful in monitoring fetal well-being in fetuses with CCHB. STUDY DESIGN: We reviewed data on all patients diagnosed prenatally with autoimmune-mediated CCHB at our institution over the past 2 years. The fetal echocardiograms from each patient were analyzed for: CPR, UA-PI, MCA-PI, cardiothoracic ratio, degree of tricuspid regurgitation, ventricular function and presence of effusions. We compared hemodynamic data from the fetal echocardiogram before delivery in patients who required urgent pacemaker placement postnatally to those patients that did not require an urgent pacemaker. RESULT: Five patients with autoimmune-mediated CCHB were identified, and two patients required urgent pacemaker placement. The CPR before delivery was significantly lower in the patients who required urgent pacemaker placement (0.97+/-0.06 versus 1.45+/-0.03; P=0.003), but there was no difference in other fetal echocardiographic markers between groups. Both patients requiring urgent pacemaker placement had a CPR less than 1.08. CONCLUSION: The CPR is predictive of postnatal outcome in fetuses with CCHB and is useful in prenatal monitoring of these patients.

A case of an infant with flail tricuspid valve due to spontaneous papillary muscle rupture: was neonatal lupus the culprit?

A 3-month-old infant presented in extremis with a flail tricuspid valve. The authors theorized that the genesis of her papillary muscle rupture was perinatal ischemia compounded by worsening pulmonary valvular stenosis leading to excessive fiber tension. Her underlying diagnosis of autoimmune-mediated heart block with endocardial fibroelastosis and prenatal glucocorticoid steroid treatment represents potentiating factors.

Isolated infundibuloarterial inversion.

Isolated infundibuloarterial inversion is a rare conotruncal cardiac anomaly characterized by a leftward and posterior aortic valve in the face of ventriculoarterial concordance. It has previously been described only in cases associated with severe additional defects of substantial hemodynamic significance. We present a case of isolated infundibuloarterial inversion with a small, hemodynamically insignificant ventricular septal defect. The nature of this lesion, diagnostic methods of imaging, and features aiding its discrimination from the more commonly recognized anatomically corrected malposition of the great arteries are discussed.

Doppler echocardiography with extended transesophageal atrial pacing: predicting the efficacy of permanent atrial pacing in the patient with a small left ventricle and sinus node dysfunction.

A 2100-g neonate underwent a two-ventricular surgical repair of a right ventricle-dominant unbalanced atrioventricular septal defect associated with the heterotaxy syndrome and sinus node dysfunction. Postoperative congestive heart failure persisted despite bradycardia management by temporary ventricular pacing. Spectral Doppler echocardiographic analysis of pulmonary venous inflow and aortic outflow patterns demonstrated significant improvement with transesophageal atrial pacing. Extended transesophageal pacing was performed for two days, resulting in dramatic clinical improvement. This is the first report of extended transesophageal atrial pacing complementing Doppler echocardiography predicting an improved outcome with permanent atrial pacing.

Arabinoxylans from rye and wheat seed that interact with ice.

Arabinoxylans that interfere with growth of ice crystals have been purified from rye (Secale cereale L., Rosen) and two varieties of wheat (Triticum aestivum L., Genesee and Hillsdale) seed. The most active polysaccharide from each seed type was homogeneous in the sense that all the molecules were in the same size range, they contained the same sugar residues, and they reacted similarly in chemical characterization experiments. Structural studies showed that the polysaccharides consist of a xylan chain to which are attached side-chains that contain a single, terminal arabinose residue. The polysaccharides differ with respect to the number of arabinose residues. The xylose:arabinose ratios in the most active fractions from rye, Genesee wheat, and Hillsdale wheat are 1.26, 1.54, and 2.08, respectively. Gel-permeation column chromatography showed that the most active polysaccharide from each seed type has a molecular weight greater than 2 x 10(6) and that the rye polysaccharide is slightly larger than the Hillsdale wheat polysaccharide. The rye polysaccharide is a better inhibitor of ice-crystal growth than is the Hillsdale wheat polysaccharide.