UNASSIGNED: Complete repair (CR) can be delayed in neonates with symptomatic tetralogy of Fallot (sTOF) using surgical or transcatheter palliation to relieve cyanosis. Balloon pulmonary valvuloplasty (BPV) is an established treatment for pulmonary valve stenosis; however, its effectiveness in palliating neonates with sTOF has not been well investigated.
UNASSIGNED: A retrospective chart review between 2005 and 2017 on neonates with sTOF who underwent initial BPV from 9 participating centers of the Congenital Cardiac Research Collaborative was performed. Primary outcome was CR at >30 days after BPV without interval reintervention (RI).
UNASSIGNED: In total, 47 neonates with sTOF underwent BPV, of whom 27 (57%) underwent CR at >30 days after BPV without RI. The median time to CR was 151 days (106-210). RI before CR occurred in 17 patients (36%): surgical shunt (n = 7), outflow tract stenting (n = 6), patent ductus arteriosus stenting (n = 2), and surgical outflow patch (n = 2). Valve-sparing repair at CR was performed in 6 patients (13%) after initial BPV. RI or CR ≤30 days from BPV was associated with smaller infundibular diastolic diameter (P = .004). An infundibular diastolic diameter of <3.4 mm demonstrated 75% sensitivity and 67% specificity to predict early CR or RI.
UNASSIGNED: BPV can be an effective palliative therapy in select neonates with sTOF to delay CR. A smaller diastolic infundibulum diameter is a predictor of RI or early CR, and valve-sparing repair is uncommon, making patient selection and alternative palliative methods key when considering BPV palliation in neonates with sTOF.

METHODS: A 36-year-old woman with a medical history of opioid use disorder and frequent urinary tract infections presented to the ED from her opioid use disorder clinic, where she was found to have an oxygen saturation by pulse oximetry (Spo2) of 82% on room air. Starting 3 days before presentation, the patient\'s family noted worsening pale complexion and blue lips at rest. These findings of cyanosis had occurred a few times before and always resolved within a couple days without any medical intervention. She had no pulmonary symptoms outside of long-standing dyspnea with moderate exertion when at work or doing chores around the house. Her medications included methadone 160 mg daily, acetaminophen 650 mg nightly as needed, and phenazopyridine 199 mg three times daily as needed for increased urinary frequency and urethral discomfort that lasted a maximum of 4 days at a time. She confirmed she had started taking a new course of phenazopyridine 4 days before presenting to the ED. She had no dietary restrictions, had been eating her normal diet, and lived in a mobile home with her family, two dogs, and a gerbil. The patient reported using less than 10 tobacco cigarettes per day, one marijuana cigarette nightly, and no alcohol or other drugs. She worked in a warehouse stacking prepackaged bread.

A pregnant female in her early 30s presented with cyanosis and oxygen saturation of 78%. She ingested isopropyl nitrate mistaking it for cannabidiol. Her arterial blood gas showed a methaemoglobin of >30% (outside the measuring range). She was treated with 120 mg of methylthioninium chloride (2 mg/kg) and symptoms improved. Her pregnancy progressed but was induced at 36 weeks because her child was small for gestational age. Methaemoglobinaemia is a rare presentation in pregnancy. There have been no reported cases of isopropyl nitrate-induced methaemoglobinaemia in pregnancy. Historically, intra-amniotic methylthioninium chloride was used in amniocentesis but use stopped after links to fetal malformations and neonatal death were made. There is no evidence outlining the risks of isopropyl nitrate in pregnancy and limited data on fetal effects from maternal exposure to intravenous methylthioninium chloride. This case adds to the evidence that treating methaemoglobinaemia may outweigh the risks of maternal exposure to methylthioninium chloride.

BACKGROUND: Absent Pulmonary Valve Syndrome (APVS) is a rare birth defect where the pulmonary valve is missing or underdeveloped. APVS often occurs alongside Tetralogy of Fallot, (TOF) another heart defect.
METHODS: A 33-year-old woman gave birth to a male infant with severe pulmonary stenosis (PS) and a large ventricular septal defect (VSD). The infant underwent surgery to close the VSD and resect the stenotic ring. Two years later, he remained asymptomatic with a closed VSD and no pulmonary valve gradient.
CONCLUSIONS: Despite high mortality rates, long-term survival has improved with advancements in surgical repair. This case underscores the significance of early detection and personalized surgical strategies for complex congenital heart defects.
CONCLUSIONS: Early identification of subtle symptoms is crucial for timely intervention, while individualized surgical strategies optimize outcomes. Further research is needed to understand the complex interplay of cardiac anomalies in APVS, particularly the absence of a patent ductus arteriosus in this case.

Hypercyanotic spells are one of the defining clinical features of Tetralogy of Fallot (TOF). Limited data exist on peak Doppler right ventricular outflow tract (RVOT) gradient as a risk factor for the development of hypercyanotic spells, frequency of prophylactic use of propranolol based on peak RVOT gradient, and its impact on preventing the occurrence of hypercyanotic spells. We aimed to quantify peak RVOT gradients as measured on transthoracic echocardiography in infants with unrepaired TOF and assess for correlation with clinical symptoms of hypercyanotic spells. We also assessed the frequency of pre-operative use of propranolol, indication for medication initiation, and occurrence of hypercyanotic spells with or without propranolol use. Retrospective analysis was performed on patients at our institution who were born between February 1, 2011 and May 31, 2023. Patients were excluded if they were maintained on prostaglandin infusion or underwent palliative shunt placement or balloon valvuloplasty prior to complete surgical repair. Demographics, occurrence of hypercyanotic spells, propranolol use, peripheral oxygen saturation, age at surgical repair, and peak RVOT gradient at the time of propranolol initiation were collected from the electronic medical record. If no propranolol use was recorded, the single highest maximum RVOT gradient prior to surgery was collected. 203 patients were identified, of which 92 patients were included in analysis. Thirty-six (39%) patients received propranolol and 19% of patients developed hypercyanotic spells prior to surgery. Patients with higher peak RVOT gradients were more likely to be started on propranolol even in the absence of overt symptoms, and they also demonstrated more systemic desaturation. Additionally, peak RVOT gradient was found to be a poor predictor for the development of hypercyanotic spells. Wide clinical variation exists in the prophylactic use of propranolol for prevention of hypercyanotic spells. Peak RVOT gradient is not a reliable tool for prophylactic propranolol initiation to prevent hypercyanotic spells.

Methemoglobinemia is a rare dyshemoglobin disorder which can either be congenital or acquired. Dyshemoglobin disorders can be asymptomatic or symptomatic. We narrate the case of a 12-year-old girl who presented with a fever, cough, and oxygen saturation of 85%. She was diagnosed with COVID-19, along with a large atrial septal defect and pulmonary arterial hypertension. Arterial blood gas analysis revealed normal partial pressure of oxygen and on 100% exposure to oxygen, blood color turned chocolate brown. After the resolution of COVID-19 in 10 days, the patient was treated with oral ascorbic acid and successful atrial septal defect repair. It is important to suspect dyshemoglobin disorder in a patient who presents with hypoxia/hypoxemia.

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Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with other types of CHD (such as heterotaxy or HLHS). TAPVR is defined as an abnormal connection where all pulmonary veins do not drain into the left atrium but into the right atrium either directly or through a vein that is connected to the right atrium. TAPVR can be divided into four anatomic groups (Fig. 32.1): (1) supracardiac (about 55%), (2) cardiac (about 30%), (3) infracardiac (about 13%), and (4) mixed (very rare). In addition, it can be divided into two physiological types: nonobstructed and obstructed. Embryologically, all pulmonary veins usually connect to a pulmonary venous confluence that connects to the left atrium. If this connection does not occur, the pulmonary venous confluence connects to a systemic vein instead.

Ventricular septal defects (VSDs) occur in 1.5-3.5 of 1000 live births and constitutes 20 % of congenital cardiac defects. There is no gender predominance.