Change in biventricular function after cone reconstruction of Ebstein's anomaly: an echocardiographic study.

Aims: The Cone reconstruction in Ebstein's anomaly (EA) aims to reduce tricuspid valve regurgitation (TR) and reposition the valve to the anatomic annulus, but post-operative progress of ventricular function is poorly understood. This study evaluated biventricular function after Cone reconstruction using echocardiographic techniques. Methods and results: A retrospective study assessing longitudinal change was conducted from 2009 to 2014. All symptomatic patients with EA and severe TR undergoing surgery were included. Transthoracic advanced echocardiography was performed pre- and post-operatively (at short-term (<30 days) and mid-term). Conventional and longitudinal 2D strain parameters were measured for left ventricle (LV) and right ventricle (RV). Paired analyses were compared using Wilcoxon Matched-pairs signed rank test. From the 38 patients operated for EA, the echocardiographic data of 17 patients, aged 15 (1-57 years) at operation could be analysed. Median follow up was 6 months (8 days-54 months). The tricuspid annular plane systolic excursion (26.42 ± 5.79 mm vs. 8.75 ± 3.18 mm, P < 0.001), RV fractional area change (FAC) (45.00 ± 8.13% vs. 35.46 ± 5.76%, P = 0.038) and LV 2D peak systolic strain were significantly reduced post-operatively (-20.49 ± 2.79 vs. -17.73 ± 2.76, P = 0.041), with a trend to later recovery for LV 2D strain. There was no evidence of systolic mechanical dys-synchrony before or after operation. Conclusion: Although clinical outcome of Cone reconstruction for EA remains excellent, acute post-operative changes leads to reduction of myocardial function of both ventricles, with a trend to later recovery for LV. Continuing impairment of RV function is multifactorial but may reflect intrinsic myocardial deficiency.

The neonatal but not the mature heart adapts to acute tachycardia by beneficial modification of the force-frequency relationship.

The force-frequency relationship (FFR) reflects alterations in intracellular calcium cycling during changing heart rate (HR). Tachycardia-induced heart failure is associated with depletion of intracellular calcium. We hypothesized (1) that the relative resistance to tachycardia-induced heart failure seen in neonatal pigs is related to differences in calcium cycling, resulting in different FFR responses and (2) that pretreatment with digoxin to increase intracellular calcium would modifies these changes. LV +dP/dt was measured during incremental right atrial pacing in 16 neonatal and 14 adult pigs. FFR was measured as the change in +dP/dt as HR was increased. Animals were randomized to control or intravenous bolus digoxin (n = 8 neonate pigs in the 0.05 mg/kg group and n = 7 adult pigs in the 0.025 mg/kg group) and paced for 90 min at 25 bpm greater than the rate of peak +dP/dt. Repeat FFR was then obtained. The postpacing FFR in neonatal control pigs shifted rightward, with peak force occurring 30 bpm greater than baseline (P < 0.03). There was no vertical shift; thus, force at 150 bpm decreased (P < 0.03) and force at 300 beats/min increased (P < 0.08). In adult control pigs, FFR shifted downward (P < 0.01), with decreased force generation at all HRs. In both neonates and adult pigs, digoxin increased +dP/dt at all HRs; however, in neonate pigs digoxin decreased the contractile reserve by abrogation of the rightward shift of FFR. An adaptive response to tachycardia in the neonate pig leads to improved force generation at greater HRs. Conversely, the response of the mature pig heart is maladaptive with decreased force generation. Pretreatment with digoxin modifies these responses.

Simulation of congenital heart defects: a novel way of training in echocardiography.

BACKGROUND: Echocardiography is one of the most important diagnostic imaging modalities in paediatric cardiology. Owing to the large number of lesions, achieving expertise often requires years of training. Echocardiography is still taught using the apprenticeship model, which is time- and personnel consuming. OBJECTIVES: To extend the echocardiography simulator EchoCom to enable simulation of congenital heart lesions and validate it for training in paediatric echocardiography. METHODS: The simulator consists of a life-size manikin, a dummy transducer with attached three-dimensional (3D) tracking system and a computer application. Transthoracic real-time (RT) 3D echocardiographic datasets were collected and embedded into the simulator. Two-dimensional images were calculated and resliced from these datasets according to the position of the tracking sensor. Ten RT 3D datasets of congenital heart lesions were selected for validation. Datasets were blinded and without additional information presented to 43 participants who were stratified according to their expertise (12 experts, 16 intermediates, 15 beginners). Participants were asked to list the relevant findings and make a diagnosis. Construct validation was tested comparing diagnostic performance for each group. Face and content validation were tested using a standardised questionnaire. RESULTS: Participants judged the simulator as realistic and useful. The main drawback was the adult size of the manikin. The diagnostic performance of each group differed significantly proving construct validity. CONCLUSIONS: According to this validation the prototype simulator could make a significant contribution to training in the use of echocardiography in congenital heart disease.

Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation.

BACKGROUND: The pulsatile nature of pulmonary blood flow is important for shear stress-mediated release of endothelium-derived nitric oxide (NO) and lowering pulmonary vascular resistance (PVR) by passive recruitment of capillaries. Normal pulsatile flow is lost or markedly attenuated after Fontan-type operations, but to date, there are no data on basal pulmonary vascular resistance and its responsiveness to exogenous NO at late follow-up in these patients. METHODS AND RESULTS: We measured indexed PVR (PVRI) using Fick principle to calculate pulmonary blood flow, with respiratory mass spectrometry to measure oxygen consumption, in 15 patients (median age, 12 years; range, 7 to 17 years; 12 male, 3 female) at a median of 9 years after a Fontan-type operation (6 atriopulmonary connections, 7 lateral tunnels, 2 extracardiac conduits). The basal PVRI was 2.11+/-0.79 Wood unit (WU) times m2 (mean+/-SD) and showed a significant reduction to 1.61+/-0.48 (P=0.016) after 20 ppm of NO for 10 minutes. The patients with nonpulsatile group in the pulmonary circulation dropped the PVRI from 2.18+/-0.34 to 1.82+/-0.55 (P<0.05) after NO inhalation. CONCLUSIONS: PVR falls with exogenous NO late after Fontan-type operation. These data suggest pulmonary endothelial dysfunction, related in some part to lack of pulsatility in the pulmonary circulation because of altered flow characteristics. Therapeutic strategies to enhance pulmonary endothelial NO release may have a role in these patients.

Percutaneous implantation of pulmonary valves.

Percutaneous implantation of valves is evolving as the most exciting innovation in transcatheter therapeutics. Valve repairs and replacements form an important indication of re-operations after congenital heart operations. Right ventricular outflow tract surgery is common after repair of conditions such as pulmonary atresia with Tetralogy of Fallot, common arterial trunk and Rastelli operation for transposition of great arteries, ventricular septal defect with pulmonary stenosis. Conduit survival is improving, nevertheless, re-operations are common. Although the re-operations can be performed at low risk, they still involve morbidity and mortality. Percutaneous implantation of valves is emerging as an alternative option for these group of patients. Our experience in percutaneous implantation of pulmonary valves has yielded encouraging results. For incompetence, the results are excellent. Evolving technology is making it applicable in wider clinical situations. Similar technology could also be applied to implantation of valves in the aortic position.

Effect of fenestration on the sub-diaphragmatic venous hemodynamics in the total-cavopulmonary connection.

OBJECTIVE: To understand differences in the sub-diaphragmatic venous physiology between patients with fenestrated and non-fenestrated total-cavopulmonary connections (TCPC). METHODS: We studied the effects of respiration, retrograde flow, and gravity on the sub-diaphragmatic venous flows in 20 normal healthy volunteers (control), 25 Fontan patients with non-fenestrated TCPC, and 21 with fenestrated TCPC. Subhepatic inferior vena cava (IVC), hepatic vein (HV), and portal vein (PV) flow rates were measured with Doppler ultrasonography during inspiration and expiration in both supine and upright positions. The supine inspiratory-to-expiratory flow rate ratio was calculated to reflect the effect of respiration, the supine-to-upright flow rate ratio was calculated to assess the effect of gravity, and the magnitude of retrograde flow was evaluated with respect to total antegrade flow. Mean IVC, HV, and wedged hepatic venous (WHV) pressures were measured during cardiac catheterization in four TCPC patients before and after fenestration closure. The transhepatic venous pressure gradient (TVPG) was calculated as the difference between the HV and WHV pressure. RESULTS: Compared with control, HV flow in TCPC was heavily dependent on respiration; this inspiratory capacity was greater in fenestrated than non-fenestrated subjects (inspiratory-to-expiratory flow ratio 1.7, 4.4, and 3.0, respectively P<0.001). Normal retrograde HV flow was diminished in TCPC patients, furthermore, fenestrated subjects had less flow reversal than non-fenestrated (retrograde as percent of antegrade flow 43, 19, and 30%, respectively P<0.001). Gravity decreased IVC and HV flows more in TCPC subjects than control, but this effect was not different between the two TCPC groups. Closure of the fenestration resulted in higher IVC and HV pressures (pre-closure versus post-closure pressures [mmHg]: 11.2 +/- 4.0 vs. 12.3 +/- 3.9, and 11.5 +/- 3.8 vs. 12.4 +/- 3.8, respectively P< or =0.001). The normal TVPG was reduced in fenestrated TCPC, and worsened after fenestration closure (0.9 +/- 0.3 and 0.7 +/- 0.4, respectively P < 0.04). CONCLUSIONS: Fenestration of the inferior venous connection has important influences on sub-diaphragmatic venous return in TCPC patients. Although fenestration lowers venous pressures and partially restores TVPG, its beneficial effects on flow in TCPC patients are mediated primarily by an increase in inspiration-derived forward HV flow and reduced flow reversal. These observations suggest fenestration results in a more efficient and less congested splanchnic circulation in TCPC patients, and may have important implications in the early and late management of Fontan patients.

Subdiaphragmatic venous hemodynamics in the Fontan circulation.

OBJECTIVE: We investigated the subdiaphragmatic venous physiology in patients subjected to the Fontan operation to understand some of the early and late problems of this circulation. METHODS: Flows were evaluated by Doppler ultrasonography in the subhepatic inferior vena cava, hepatic vein, and portal vein during respiratory monitoring and with a tilt table. Twenty control subjects (group A) and 56 patients who had the Fontan operation, 27 in functional class I (group B) and 29 in class III or IV (group C), were studied. Inspiratory/expiratory flow ratio was calculated to reflect respiratory effects, and upright/supine flow ratio was calculated to assess gravity effects. Inferior vena caval, hepatic venous, and wedged hepatic venous pressures were measured during catheterization in 21 control subjects and 25 Fontan patients. The difference between wedged and hepatic venous pressures represents the transhepatic venous pressure gradient. RESULTS: Fontan hepatic venous flow depended more on inspiration than control, but without difference between groups B and C (inspiratory/expiratory flow ratios: 1.7, 2.9, and 2.9, respectively; P <.02). Normal portal venous flow was higher in expiration; this effect was lost in group B and reversed in group C (inspiratory/expiratory flow ratios: 0.8, 1.0, and 1.3; P <.0005). Gravity reduced portal venous flow in groups A and B, but progression to functional class III or IV (group C) exacerbated this effect (upright/supine flow ratios: 0.8, 0.7, and 0.5; P <.01). Inferior vena caval, hepatic venous, and wedged hepatic venous pressures (in millimeters of mercury) in the Fontan groups were all elevated compared with the control group (inferior vena cava, 14.4 +/- 4.4 vs 5.9 +/- 2.3; hepatic vein, 14.7 +/- 4.5 vs 5.9 +/- 1.9; wedged hepatic vein, 14.7 +/- 4.0 vs 8.3 +/- 2.6; P <.0001). However, transhepatic venous pressure gradient in the Fontan group was lower than in the control group (0.5 +/- 0.5 vs 2.4 +/- 2.0; P <.001). Univariate analysis of inferior vena caval pressure and transhepatic venous pressure gradient showed significant inverse correlation (r = 0.6, P <.002). CONCLUSIONS: In patients who are in functionally poorer condition after the Fontan operation, portal venous flow loses normal expiratory augmentation and adverse gravity influence is enhanced. These suboptimal flow dynamics, coupled with higher splanchnic venous pressures and lower transhepatic venous pressure gradients, suggest that hepatic sinusoids are congested, acting as "open tubes." Transhepatic gradient loss is incrementally worse with higher caval pressures. These observations may be responsible for late gastrointestinal problems in patients who have had the Fontan operation.

Effects of respiration and gravity on infradiaphragmatic venous flow in normal and Fontan patients.

BACKGROUND: In the Fontan circulation, pulmonary and systemic vascular resistances are in series. The implications of this unique arrangement on infradiaphragmatic venous physiology are poorly understood. METHODS AND RESULTS: We studied the effects of respiration and gravity on infradiaphragmatic venous flows in 20 normal healthy volunteers (control) and 48 Fontan patients (atriopulmonary connection [APC] n=15, total cavopulmonary connection [TCPC] n=30). Hepatic venous (HV), subhepatic inferior vena caval (IVC), and portal venous (PV) flow rates were measured with Doppler ultrasonography during inspiration and expiration in both the supine and upright positions. The inspiratory-to-expiratory flow rate ratio was calculated to reflect the effect of respiration, and the supine-to-upright flow rate ratio was calculated to assess the effect of gravity. HV flow depended heavily on inspiration in TCPC compared with both control and APC subjects (inspiratory-to-expiratory flow rate ratio 3.4, 1.7, and 1.6, respectively; P:<0.0001). Normal PV flow was higher in expiration, but this effect was lost in TCPC and APC patients (inspiratory-to-expiratory flow rate ratio 0.8, 1.0, and 1.1, respectively; P:=0.01). The respiratory influence on IVC flow was the same in all groups. Gravity decreased HV flow more in APC than in TCPC patients (supine-to-upright flow rate ratio 3.2 versus 2.1, respectively; P:<0.04) but reduced PV flow equally in all groups. CONCLUSIONS: Gravity and respiration have important influences on infradiaphragmatic venous return in Fontan patients. Although gravity exerts a significant detrimental effect on lower body venous return, which is more marked in APC than in TCPC patients, the beneficial effects of respiration in TCPC patients are mediated primarily by an increase in HV flow. These effects may have important short- and long-term implications for the hemodynamics of the Fontan circulation.

Scleredema adultorum.

Scleredema adultorum is a rare connective tissue disorder reported usually following streptococcal infection, influenza, measles, and mumps. It has been reported occasionally following trauma and tuberculous lymphadenitis. This is a report of scleredema adultorum developing after chicken pox in an eight-year-old male child. The diagnosis was established by characteristic picture on skin biopsy using special stain. The patient had a benign course and a spontaneous recovery in two weeks. The case has been reported as the first case of scleredema adultorum developing after chicken pox.

Common arterial trunk associated with double aortic arch.

The combination of common arterial trunk associated with double aortic arch is very rare. We are aware of only four cases ever reported in English literature. We add two cases of this entity and comment on the morphological aspects, the clinical impact of the combined lesions, and their diagnostic and therapeutic implications.

Familial congenital valvar pulmonary stenosis: autosomal dominant inheritance.

A mother and three of her four children, one girl and two boys, who had valvar pulmonary stenosis are described. One child had an associated septum secundum atrial septal defect, and another had an associated ventricular septal defect. An autosomal dominant mode of inheritance is likely for the valvar pulmonary stenosis. The association of additional cardiac lesions in two of the three siblings raises the possibility of closely associated genetic loci.

Neonatal mydriasis due to effects of atropine used for maternal Tik-20 poisoning.

A neonate was born to a mother who had consumed an organophosphorus(OPC) compound with suicidal intent. The mother was administered atropine and this caused mydriasis in the neonate without any other pharmacological effects. There was no evidence of placental dysfunction. There are no case reports of OPC consumed in pregnancy and its effect on neonates or of effects of massive doses of atropine in the mother and its effects on the fetus or the newborn.

Jejunal disaccharidases in protein energy malnutrition and recovery.

The jejunal disaccharidases, sucrase, maltase and lactase, were determined in jejunal biopsies obtained from 43 malnourished children and 10 controls. In the study group, 63% were girls and 93% had severe malnutrition. Lactase activity was significantly reduced in third and fourth degree malnutrition (p < 0.05 and p < 0.005, respectively), but maltase activity was significantly reduced only in the fourth degree malnutrition (p < 0.01). After recovery, maltase and sucrase activities showed a marginally significant increase (p = 0.06), where lactase showed no significant increase (p > 0.05). We conclude that jejunal disaccharidase activity decreases significantly with increasing severity of malnutrition, lactase being the most severely affected and the last to recover.

Bacteriological profile of neonatal septicemia cases (for the year 1990-91).

Blood culture reports were studied in 1266 cases of clinically suspected neonatal septicemia, to determine the bacteriological profile and antibiotic sensitivity pattern of the cultured isolates. Blood culture was positive in 24.88% of cases. Gram negative septicemia was encountered in 87.1% of these neonates. Klebsiella and Enterobacter species were the predominant pathogens amongst Gram negative organisms. Of Gram positive isolates, Staphylococcus aureus was the predominant isolate (79.0%). Salmonella species was isolated in 2.4% of these cases.

Spondylocostal dysostosis.

Spondylocostal dysostosis is a rare condition characterized by short stature due to a short trunk, multiple morphological abnormalities of the vertebrae and ribs due to malsegmentation of the axial skeleton. Radiological features include reduced number of vertebrae and ribs, hemivertebrae, fused or sagitally cleft vertebrae or multiple rib fusions. Three distinct clinical entities are described. We report nine cases of this syndrome, seven were infants and presented with an abnormal shape of the thorax. Two of them had a meningocoele, and one succumbed to the CNS anomalies on the third day of life. The other two cases were two and eight years of age. Cardiac lesion was detected in one case and renal malformations in three cases. Reduction in the rib number was present in all cases, and rib fusion in seven cases. Thoracic vertebral dysegmentation was noted in all, lumbar in three and cervical in one case. This is the first large series from India. No clear single etiology was established.