Abstract Interventional cardiology plays a key role in the diagnosis and management of patients with functionally univentricular physiology after the various stages of surgical palliation. The interventions performed are widely variable in type, including angioplasty of stenotic vessels and implantation of stents in stenotic vessels; closure of defects such as collaterals, leaks in baffles, and fenestrations; creation of fenestration; and more. In the setting of venous hypertension associated with stenosis at the Fontan baffle, conduit, or pulmonary arteries, stent implantation is often preferred, as the aim is to eliminate completely the narrowing, given that relatively mild stenosis can have a significant detrimental hemodynamic effect in patients with functionally univentricular circulation. The procedure is highly successful. In patients who fail after Fontan procedure, creation of a fenestration is often performed, with variable technique depending on the underlying anatomic substrate. To increase chances of patency of the fenestration, implantation of a stent is often required, particularly in the setting of an extracardiac conduit. For those patients with cyanosis and favorable Fontan hemodynamics, closure of the fenestration is performed using atrial septal occluder devices with high success rate. Coils compatible with magnetic resonance imaging are used widely to treat collateral vessels, although on occasion other specific embolization tools are required, such as particles or vascular plugs. Postoperative arch obstruction is successfully managed with angioplasty at a younger age, while implantation of a stent in the aorta is reserved for older patients. Specifics of these interventional procedures as applied to the population of patients with functionally univentricular hearts are described in this manuscript.
Coarctation of the aorta (CoA) is a relatively common congenital cardiac defect often causing few symptoms and therefore can be challenging to diagnose. The hallmark finding on physical examination is upper extremity hypertension, and for this reason, CoA should be considered in any young hypertensive patient, justifying measurement of lower extremity blood pressure at least once in these individuals. The presence of a significant pressure gradient between the arms and legs is highly suggestive of the diagnosis. Early diagnosis and treatment are important as long-term data consistently demonstrate that patients with CoA have a reduced life expectancy and increased risk of cardiovascular complications. Surgical repair has traditionally been the mainstay of therapy for correction, although advances in endovascular technology with covered stents or stent grafts permit nonsurgical approaches for the management of older children and adults with native CoA and complications. Persistent hypertension and vascular dysfunction can lead to an increased risk of coronary disease, which, remains the greatest cause of long-term mortality. Thus, blood pressure control and periodic reassessment with transthoracic echocardiography and three-dimensional imaging (computed tomography or cardiac magnetic resonance) for should be performed regularly as cardiovascular complications may occur decades after the intervention.
Abstract Infants with dextro-transposition of the great arteries (d-TGA) are at high risk for hemodynamic compromise in the immediate postnatal period due to dependence on intracardiac mixing for oxygen delivery. This period of profound hypoxemia may have long-term implications, as previous studies demonstrated patients with d-TGA are at increased risk for neurocognitive delays despite effective surgical correction in the neonatal period. Balloon atrial septostomy (BAS) is an established intervention that improves intracardiac mixing and perioperative hemodynamics. This retrospective study aimed to quantify the time from birth to BAS and compare short-term outcomes for patients with prenatal and postnatal diagnoses of d-TGA. We identified 68 newborns born with d-TGA who were admitted to our facility between 2013 and 2022 and required BAS within 48 hours after birth. A prenatal diagnosis significantly decreased the time from birth to BAS (9.3 to 2.8 hours, p-value < 0.0001), however, there was no significant difference in other short-term outcomes including lowest SpO2, initial lactate, neurologic complications, time to surgery, or hospital length of stay. During this study, our cardiac interventional team began traveling to a nearby delivery center where a bedside BAS could be performed prior to transferring the patient. This proved to be a safe and effective model, as there were no procedural complications and infants with a prenatal diagnosis born after program implementation had a significantly reduced time to BAS (4.1 to 1.1 hours, p-value < 0.0001).
