Atrial Septal Defect Closure: Not Always Straightforward.

A nine-year old boy presented following a chest x-ray for COVID-19 infection that showed cardiomegaly. Transthoracic echocardiogram revealed a large atrial septal defect, dilated right heart, and normal function. Surgical repair was performed eight weeks after his COVID diagnosis. After weaning from cardiopulmonary bypass, pulmonary artery pressures were 2/3 systemic and ectopy was seen, requiring inotropes, nitric oxide, lidocaine, and amiodarone. He was discharged on postoperative day (POD) 5. On POD 6, he presented with acute right foot ischemia. Computed tomography showed a large aortic thrombus, requiring emergent thrombectomy. Coagulopathy workup was negative. Cardiac magnetic resonance imaging (CMR) and catheterization showed reduced biventricular function and diastolic dysfunction. Diuretics and ß blockers were started, with gradual improvement in left ventricular systolic function.

Regionalization or Access to Care? A Joint Pediatric Heart Care Program That Achieves Both: One Program-Two Sites.

Background: Regionalization of care for children with congenital heart disease has been proposed as a method to improve outcomes. This has raised concerns about limiting access to care. We present the details of a joint pediatric heart care program (JPHCP) which utilized regionalization and actually improved access to care. Methods: In 2017, Kentucky Children's Hospital (KCH) launched the JPHCP with Cincinnati Children's Hospital Medical Center (CCHMC). This unique satellite model was the product of several years of planning, leading to a comprehensive strategy with shared personnel, conferences, and a robust transfer system; "one program-two sites." Results: Between March 2017 and the end of June 2022, 355 operations were performed at KCH under the auspices of the JPHCP. As of the most recent published Society of Thoracic Surgeons (STS) outcome report (through the end of June 2021), for all STAT categories, the JPHCP at KCH outperformed the STS overall in postoperative length of stay, and the mortality rate was lower than expected for the case mix. Of the 355 operations, there were 131 STAT 1, 148 STAT 2, 40 STAT 3, and 36 STAT 4 operations, with two operative mortalities: an adult undergoing surgery for Ebstein anomaly, and a premature infant who died from severe lung disease many months after aortopexy. Conclusions: With a select case mix, and by affiliating with a large volume congenital heart center, the creation of the JPHCP at KCH was able to achieve excellent congenital heart surgery results. Importantly, access to care was improved for those children at the more remote location utilizing this one program-two sites model.

Differences in Cost of Care by Palliation Strategy for Infants With Ductal-Dependent Pulmonary Blood Flow.

BACKGROUND: In infants with ductal-dependent pulmonary blood flow, initial palliation with patent ductus arteriosus (PDA) stent or modified Blalock-Taussig (BT) shunt have comparable mortality but discrepant length of stay, procedural complication rates and reintervention burdens, which may influence cost. The relative economic impact of these palliation strategies is unknown. METHODS AND RESULTS: Retrospective study of infants with ductal-dependent pulmonary blood flow palliated with PDA stent (n=104) or BT shunt (n=251) from 2008 to 2015 at 4 centers of the Congenital Catheterization Research Collaborative. Inflation-adjusted inpatient hospital costs were calculated for first year of life using Pediatric Health Information System data. Costs derived from outpatient catheterizations not in Pediatric Health Information System were imputed. Costs were compared using propensity score-adjusted multivariable models, to account for baseline differences between groups. After propensity score adjustment, first year of life costs were significantly lower in PDA stent ($215 825 [190 644-244 333]) than BT shunt ($249 855 [230 693-270 609]) patients ( P=0.05). After addition of imputed costs, first year of life costs were not significantly different between PDA stent ($226 403 [200 274-255 941]) and BT shunt ($252 072 [232 955-272 759]) groups ( P=0.15). Patient characteristics associated with higher costs included: younger gestational age, genetic syndrome, noncardiac diagnoses, procedural complications, extracorporeal membrane oxygenation, duration of ventilation, intensive care unit and hospital length of stay and reintervention ( P≤0.02 for all). CONCLUSIONS: In this first multicenter comparative cost study of PDA stent or BT shunt as palliation for infants with ductal-dependent pulmonary blood flow, adjusted for baseline differences, PDA stent was associated with lower to equivalent costs over the first year of life. Combined with previous evidence suggesting clinical noninferiority, these findings suggest that PDA stent provides competitive health care value.

Comparison Between Patent Ductus Arteriosus Stent and Modified Blalock-Taussig Shunt as Palliation for Infants With Ductal-Dependent Pulmonary Blood Flow: Insights From the Congenital Catheterization Research Collaborative.

BACKGROUND: Infants with ductal-dependent pulmonary blood flow may undergo palliation with either a patent ductus arteriosus (PDA) stent or a modified Blalock-Taussig (BT) shunt. A balanced multicenter comparison of these 2 approaches is lacking. METHODS: Infants with ductal-dependent pulmonary blood flow palliated with either a PDA stent or a BT shunt from January 2008 to November 2015 were reviewed from the 4 member centers of the Congenital Catheterization Research Collaborative. Outcomes were compared by use of propensity score adjustment to account for baseline differences between groups. RESULTS: One hundred six patients with a PDA stent and 251 patients with a BT shunt were included. The groups differed in underlying anatomy (expected 2-ventricle circulation in 60% of PDA stents versus 45% of BT shunts; P=0.001) and presence of antegrade pulmonary blood flow (61% of PDA stents versus 38% of BT shunts; P<0.001). After propensity score adjustment, there was no difference in the hazard of the primary composite outcome of death or unplanned reintervention to treat cyanosis (hazard ratio, 0.8; 95% confidence interval [CI], 0.52-1.23; P=0.31). Other reinterventions were more common in the PDA stent group (hazard ratio, 29.8; 95% CI, 9.8-91.1; P<0.001). However, the PDA stent group had a lower adjusted intensive care unit length of stay (5.3 days [95% CI, 4.2-6.7] versus 9.19 days [95% CI, 7.9-10.6]; P<0.001), a lower risk of diuretic use at discharge (odds ratio, 0.4; 95% CI, 0.25-0.64; P<0.001) and procedural complications (odds ratio, 0.4; 95% CI, 0.2-0.77; P=0.006), and larger (152 mm2/m2 [95% CI, 132-176] versus 125 mm2/m2 [95% CI, 113-138]; P=0.029) and more symmetrical (symmetry index, 0.84 [95% CI, 0.8-0.89] versus 0.77 [95% CI, 0.75-0.8]; P=0.008] pulmonary arteries at the time of subsequent surgical repair or last follow-up. CONCLUSIONS: In this multicenter comparison of palliative PDA stent and BT shunt for infants with ductal-dependent pulmonary blood flow adjusted for differences in patient factors, there was no difference in the primary end point, death or unplanned reintervention to treat cyanosis. However, other markers of morbidity and pulmonary artery size favored the PDA stent group, supporting PDA stent as a reasonable alternative to BT shunt in select patients.

Effect of hypertension on reproductive organ weights in various strains of rats.

The testes of Wistar Kyoto (WKY) and Spontaneously Hypertensive (SHR) rats have been shown to have differences in regional vascular resistance. In addition, myocardial hypertrophy tends to be more pronounced and occur more frequently in WKY and SHR female rats than in their male counterparts. Therefore, we sought to determine whether hypertension had any effect on reproductive organs and whether this effect was the same among strains. We removed the testes or uterus from Sprague Dawley (SD; n = 30 male and 30 female), WKY (n = 23 male and 16 female), and SHR (n = 27 male and 34 female) rats, weighed these animals and organs, and calculated the reproductive organ:body weight ratio (mean +/- 1 standard deviation x 10-3 g). The testes:body weight ratio of WKY (6.5 +/- 0.8) animals was significantly (P= 0.05) less than those of SD (7.6 +/- 1.1) and SHR (8.0 +/- 0.5) rats. The uterus:body weight ratio of the SD rats (1.7 +/- 0.4) was significantly (P = 0.05) less than those of WKY (2.4 +/- 0.6) and SHR (2.3 +/- 0.6) rats. Compared with WKY rats, male SD rats had a higher testes:body weight ratio whereas female SD rats had a lower uterus: body weight ratio. Whereas the SHR testes:body weight ratio was significantly higher than that of WKY rats, this effect was not seen for the uterus: body weight ratio. The effect of hypertension on reproductive organs should be taken into consideration when choosing a species, gender, or organ for study. As well, data compared across genders or strains must be evaluated carefully to ensure valid comparisons.

Preischemic administration of ribose to delay the onset of irreversible ischemic injury and improve function: studies in normal and hypertrophied hearts.

Compared with normal hearts, those with pathology (hypertrophy) are less tolerant of metabolic stresses such as ischemia. Pharmacologic intervention administered prior to such stress could provide significant protection. This study determined, firstly, whether the pentose sugar ribose, previously shown to improve postischemic recovery of energy stores and function, protects against ischemia when administered as a pretreatment. Secondly, the efficacy of this same pretreatment protocol was determined in hearts with pathology (hypertrophy). For study 1, Sprague-Dawley rats received equal volumes of either vehicle (bolus i.v. saline) or ribose (100 mg/kg) before global myocardial ischemia. In study 2, spontaneously hypertensive rats (SHR; blood pressure approximately 200/130) with myocardial hypertrophy underwent the same treatment protocol and assessments. In vivo left ventricular function was measured and myocardial metabolites and tolerance to ischemia were assessed. In normal hearts, ribose pretreatment significantly elevated the heart's energy stores (glycogen), and delayed the onset of irreversible ischemic injury by 25%. However, in vivo ventricular relaxation was reduced by 41% in the ribose group. In SHR, ribose pretreatment did not produce significant elevations in the heart's energy or improvements in tolerance to global ischemia, but significantly improved ventricular function (maximal rate of pressure rise (+dP/dt(max)), 25%; normalized contractility ((+dP/dt)/P), 13%) despite no change in hemodynamics. Thus, administration of ribose in advance of global myocardial ischemia does provide metabolic benefit in normal hearts. However, in hypertrophied hearts, ribose did not affect ischemic tolerance but improved ventricular function.

Body weight and food intake profiles are modulated by sex hormones and tamoxifen in chronically hypertensive rats.

Sex hormones and the selective estrogen receptor modulator tamoxifen affect food consumption and body weight in normotensive rats. This study investigated the effects of hormone manipulation and tamoxifen on weight gain and food intake in the presence of chronic systemic hypertension. Male and female spontaneously hypertensive rats (SHR) were either neutered or sham operated before puberty, and subgroups of neutered females received either estrogen replacement therapy (ERT) or tamoxifen at the age of 12 wk. Weekly body weight and food consumption were assessed, and food consumption was normalized to metabolic weight (g body(2/3)). Neutering reduced weight gain in males (P = 0.0001), but increased it in females (P < 0.0001). Both ERT and tamoxifen treatment prevented this increase in weight, with body weight dropping to levels of sham-operated rats for ERT, whereas rats given tamoxifen maintained greater body weights than sham-operated rats (P < 0.0001). This contrasts with previous work in normotensive females in which sham-operated and tamoxifen-treated females did not differ. Neutering reduced normalized food consumption relative to sham-operated rats in both males and females (P < 0.05). Although ERT returned it to normalized intakes of sham-operated rats, tamoxifen reduced normalized food consumption relative to that of both sham-operated and ERT groups. In hypertensive rats, body weight is modulated by sex hormones in both males and females, but in opposite directions. Both estrogen and tamoxifen exert immediate effects in females. Interestingly, the effect of tamoxifen on body weight appears to be greater in hypertensive than in normotensive rats.

Special Feeding and Care of Senescent Spontaneously Hypertensive Rats.

This study investigated the impact of feeding methods on body weight of senescent female spontaneously hypertensive rats (SHRs) and showed that supplementing powdered feed was useful as they approached heart failure at 22 to 23 months of age. SHRs are genetically predisposed to systemic hypertension and will, with age, progress into complete heart failure resulting in death. Close to the time of heart failure, some rats experienced a loss of appetite and weight loss. It was postulated that either elevated blood pressure, age-associated health issues, or the effort required to access pelleted food prevented the rats from eating properly, resulting in malnutrition and weight loss. As they aged, the rats benefited from the addition of powdered food to ensure that body weights remained stable and to prevent malnutrition that could lead to premature death. Animals were fed commercially available rat chow pellets until they showed persistent signs of weight loss or a lack of interest in their food. At that time, the rats were also given powdered rat chow in shallow bowls to facilitate the eating and the digestion of their food. The rats were weighed weekly to confirm they consumed sufficient calories daily and to ensure that the change to the powdered chow was having the desired effect. Prior to being fed the powdered rat chow, the rats had shown signs of progressive weight loss. After starting the powdered chow, the rats either maintained or gained weight. This study shows that as the female SHR matures, special care and handling is key to maintaining body weights and good health. With only modest changes in routine (i.e., powdered food) and an attentive eye on the rats' daily activities, it was possible to maintain these senescent female SHRs in a healthy condition until the termination of the study or onset of heart failure.