Palliative Care Referrals in Cardiac Disease.

OBJECTIVES: With evidence of benefits of pediatric palliative care (PPC) integration, we sought to characterize subspecialty PPC referral patterns and end of life (EOL) care in pediatric advanced heart disease (AHD). METHODS: In this retrospective cohort study, we compared inpatient pediatric (<21 years) deaths due to AHD in 2 separate 3-year epochs: 2007-2009 (early) and 2015-2018 (late). Demographics, disease burden, medical interventions, mode of death, and hospital charges were evaluated for temporal changes and PPC influence. RESULTS: Of 3409 early-epoch admissions, there were 110 deaths; the late epoch had 99 deaths in 4032 admissions. In the early epoch, 45 patients (1.3% admissions, 17% deaths) were referred for PPC, compared with 146 late-epoch patients (3.6% admissions, 58% deaths). Most deaths (186 [89%]) occurred in the cardiac ICU after discontinuation of life-sustaining therapy (138 [66%]). Medical therapies included ventilation (189 [90%]), inotropes (184 [88%]), cardiopulmonary resuscitation (68 [33%]), or mechanical circulatory support (67 [32%]), with no temporal difference observed. PPC involvement was associated with decreased mechanical circulatory support, ventilation, inotropes, or cardiopulmonary resuscitation at EOL, and children were more likely to be awake and be receiving enteral feeds. PPC involvement increased advance care planning, with lower hospital charges on day of death and 7 days before (respective differences $5058 [P = .02] and $25 634 [P = .02]). CONCLUSIONS: Pediatric AHD deaths are associated with high medical intensity; however, children with PPC consultation experienced substantially less invasive interventions at EOL. Further study is warranted to explore these findings and how palliative care principles can be better integrated into care.

Prenatal duct closure leading to severe pulmonary hypertension in a preterm neonate-a case report.

Prenatal closure of the ductus arteriosus (DA) can lead to cardiovascular dysfunction resulting in pulmonary hypertension (PH), progressive right heart failure, fetal hydrops, and fetal or neonatal demise. Supportive therapies-including mechanical ventilation, oxygen, and nitric oxide (NO)-have been employed with variable success among infants born full term, but there is no widely accepted management of prenatal closure of the DA, particularly for preterm infants. We present the case of an infant born at 31 weeks' gestation with right ventricular (RV) dysfunction and PH due to prenatal ductal closure, who was successfully treated with milrinone, resulting in full recovery of cardiac function. Prenatal ductal closure is rare, particularly under 32 weeks gestation, but should be suspected in cases of postnatal hypoxemia in the absence of significant lung disease or structural heart disease. Milrinone may be considered as a therapeutic agent to treat both PH and RV dysfunction in preterm infants status post in utero closure of the DA.

Congenital aortic paravalvular tunnel masquerading as a recurrent ventricular septal defect.

A 4-year-old female underwent surgical repair of an unusual variation of an aorto-left ventricular tunnel (ALVT). Serial echocardiograms had demonstrated previous spontaneous closure of a perimembranous ventricular septal defect (VSD). The patient presented with concern for residual VSD which was later demonstrated to be an eccentric jet through an ALVT. This case emphasizes early and accurate diagnosis for ALVTs and how they can be misdiagnosed as VSDs.