Patent Ductus Arteriosus

Going Home with a Patent Ductus Arteriosus: Is it Benign?

Percutaneous Closure of the Patent Ductus Arteriosus in Very Low Weight Infants: Considerations Following US Food and Drug Administration Approval of a Novel Device

The prevalence of adult congenital heart disease (ACHD) has risen markedly over the past 2 decades, with the number of adults now rivaling the number of children with severe defects.1 This is, perhaps, not surprising given that current care allows nearly 90% of infants born with heart defects to thrive into their adult years.1,2 This remarkable triumph is tempered, however, by the realization that early interventions were reparative and not curative. Numerous complications may surface years after uneventful childhood courses, justifying vigilant clinical follow-up throughout adulthood. The 12-lead ECG remains an invaluable cornerstone in the clinical appraisal of adults with congenital heart disease that, in certain circumstances, provides diagnostic and/or prognostic information. The present review imparts a clinical perspective to ECG interpretation in ACHD, emphasizing practical and pathogenomonic findings in the more frequently encountered congenital defects in adults. Anatomic features of the conduction system relevant to ECG findings in ACHD are summarized, including variations in the location of the sinus node, atrioventricular (AV) node, and His-Purkinje system. Thereafter, pertinent ECG features are highlighted for common subtypes of ACHD (Table). Examples are provided throughout for illustration. View this table: Table. Typical ECG Features in Common Forms of ACHD ### Sinus Node In the morphologically normal heart, a crescent-shaped sinus node is characteristically located epicardially along the lateral aspect of the superior cavoatrial junction. It generates a P-wave axis typically between 15° and 75°. Most patients with ACHD have normally positioned atrial chambers, called atrial situs solitus, with normal sinus node location. The position of the sinus node may, however, vary with the atrial chambers and their appendages. #### Juxtaposition of the Atrial Appendages In juxtaposition of the atrial appendages, both appendages are on the same side of the arterial pedicle rather than each being ipsilateral to its respective atrium. Left juxtaposition, with left-sided atrial appendages, frequently accompanies tricuspid atresia and has …

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When pharmacologic closure of patent ductus arteriosus (PDA) in the preterm infant using indomethacin was introduced in 1976, there was a paucity of knowledge about the factors controlling the caliber of the ductus arteriosus both before and after birth. Additionally, mortality and morbidity of prematurity (associated with what was then usually referred to as hyaline membrane disease) was remarkably high in the early 1970s. As early as the 1950s, some neonatologists recognized PDA as a complication of what later became designated as “respiratory distress syndrome” (RDS), although it was not until the advent of intermittent mandatory ventilation (IMV) and continuous positive airway pressure (CPAP) that the concept gained prominence. Patency of the ductus appeared to be a major complication, especially in the smallest of preterm babies (at that time, < 1,350 g]) and usually carried a terminal prognosis. The advent of IMV and CPAP kept these small babies alive for a sufficient period of time for the patent ductus to become recognized progressively as a distinct clinical entity and an important early complication of preterm birth. Observant neonatologists noted that babies who had RDS often improved up to a point, beyond which they worsened. The usual story was that the baby experienced ventilatory failure, was treated with mechanical ventilation, and began to improve, with lower inspired oxygen requirements. After a few days, when discussions ensued about taking the baby off the ventilator, the infant’s improvement would cease. The radiographic picture changed from a clearing of the initial ground glass appearance coupled with a relatively small heart to an enlarging heart and the pulmonary edema of congestive heart failure due to a left-to-right shunt across the PDA. Beginning in the mid-1960s, aggressive surgeons advocated operative closure of the PDA in these small preterm infants, but multiple centers consistently reported unacceptably high …

Toward a Rational Approach to Patent Ductus Arteriosus Trials: Selecting the Population of Interest

The First Closure of the Persistent Ductus Arteriosus

After completing this article, readers should be able to: 1. Describe the developments that have allowed advances in neonatal cardiac surgery. 2. Describe the diagnostic approach to hypoplastic left heart syndrome (HLHS). 3. Delineate the preoperative management of the neonate who has HLHS. 4. Describe the original Norwood procedure and subsequent modifications. 5. Delineate the optimal therapy in the postoperative period for neonates who have a single ventricle. Advances in the surgical and medical management of children who have congenital heart disease (CHD) have led to a dramatic improvement in overall survival and reduced the long-term sequelae of open-heart surgery. With improved survival and decreased morbidity over the past 2 decades, the early complete repair of complex congenital heart problems in preterm and low-birthweight neonates has gained wider acceptance. Cardiologists and cardiothoracic surgeons now consider and perform complete neonatal repair more frequently than palliative surgery in this patient population. The treatment of hypoplastic left heart syndrome (HLHS) best illustrates the evolution of management strategies to improve survival and long-term outcome. A recent modification of the Norwood operation that involves a right ventricle-to-pulmonary artery conduit has led to much improved early postoperative stability and, in many centers, improved survival to a stage II superior cavopulmonary connection. A multidisciplinary team approach is required to provide the necessary care for this complex patient population. In 1938, Robert Gross at Children’s Hospital in Boston successfully ligated a patent ductus arteriosus, and this procedure—performed against the wishes of surgeon-in-chief William Ladd—opened the era of surgery for CHD. (1) Gross and Hufnagel performed detailed animal experiments to develop a technique for treatment of coarctation of the aorta that involves excision and end-to-end anastomosis. (2) Clarence Craaford of Stockholm, Sweden, had visited Gross to observe his experimental work, and in October 1944, Craaford and Nylin were the first to repair aortic …

Transcatheter closure of patent ductus arteriosus (PDA) is a well established procedure and an accepted treatment modality for small to moderate-sized PDA. This study aimed to evaluate the immediate and follow-up results of transcatheter closure of large PDAs with severe pulmonary arterial hypertension (PAH) in adults. After a complete hemodynamic evaluation differentiating from the reversibility of severe PAH, transcatheter closure of PDA was performed. Patients were followed up clinically and echocardiographically at 24 hours, 1 month, 3 months, 6 months, 12 months and 24 months after occlusion. Twenty-nine patients had successful occlusion, pulmonary artery pressure (PAP), left ventricular ejection fraction (LVEF) and fractional shortening (FS) significantly decreased immediately after occlusion ((106 ± 25) mmHg vs. (50 ± 14) mmHg, P < 0.01; (63.7 ± 7.2)% vs. (51.4 ± 10.1)%, P < 0.01 and (36.9 ± 8.2)% vs. (28.9 ± 8.6)%, P < 0.05, respectively). At 1 month after PDA closure, the signs and symptoms improved markedly in all 29 patients, and PDAs were completely closed and remained closed during the follow-up. Eighteen patients having different degrees of dyspnea were treated with angiotensin converting enzyme inhibitor (ACEI) and/or digoxin after occlusion. Nine patients whose pulmonary vascular resistence (PVR) > 6 Wood units accepted targeted PAH therapy. After 1 to 3 months of peroral drug therapy, their exercise tolerance improved from New York Heart Association (NYHA) class III-IV to NYHA class I. During follow-up, no latent arrhythmias were found, the left atrial diameter (LAD), left ventricular end-diastolic diameter (LVEDD), left ventricular end-systolic diameter (LVESD), left ventricular mass index (LVMI) and pulmonary artery systolic pressure (PASP) decreased significantly (P < 0.05), and FS and LVEF recovered compared to the immediate postclosure state. However, FS and LVEF remained low compared to the preclosure state. Transcatheter closure of large PDA with severe PAH is feasible, effective, and safe in adults. Significant left ventricular systolic changes may occur after closure of large PDA, and left ventricular function usually recovers within a few months.

Patent Ductus Arteriosus.

Presentation of Congenital Heart Disease in the Neonate and Young Infant

After completing this article, readers should be able to: 1. Delineate the short- and long-term adverse outcomes associated with low systemic blood flow. 2. Explain why it is not appropriate to use blood pressure and population criteria for hypotension to determine which infants who have low systemic blood flow need cardiovascular support. 3. Describe the various hemodynamic states in neonates and the assessment that is useful in allowing discrimination between these states. 4. Characterize the hemodynamic effects of dopamine and dobutamine in neonates. 5. Delineate the most common characteristics of infants who fail to maintain superior vena cava flows despite the use of inotropes. Low systemic blood flow (SBF) commonly occurs in preterm infants who have respiratory distress in the first postnatal day. Such low flows are strongly associated with neonatal morbidity, including peri/intraventricular hemorrhage (P/IVH), developmental impairments, and mortality. Diagnosing which infants have low SBF is problematic; use of blood pressure (BP) and signs of poor tissue perfusion, including capillary refill times (CRT), poor renal function, and acidosis, results in substantial delays in the initiation of cardiovascular support and a proportion of affected infants missing treatment. The traditional approach to cardiovascular support of the preterm infant has been to identify infants who have hypotension and to use agents, particularly dopamine, titrated to improve BP. However, this strategy may not be the best for improving SBF, and it is yet to be demonstrated that this approach improves neonatal outcomes. This review focuses on identifying which infants have low SBF or organ blood flow, discusses mechanisms for low flows, and examines potential therapies for treating or preventing low flow. Cardiovascular compromise occurs in a number of clinical scenarios in preterm infants, but it is most common on the first postnatal day. Not all infants who have low SBF will be suspected based on clinical signs, including …

The World Pulmonary Hypertension Symposium in 2013 (Nice, France) introduced a new entity in the classification for pediatric and adult patients called “segmental pulmonary hypertension (PH).”1 Segmental PH was described in the international 2015 guidelines as PH “observed in discrete lung areas perfused by aortopulmonary collaterals in congenital heart diseases such as pulmonary or tricuspid atresia,”2 while the proceedings of the Nice World Symposium1 defined this as “PH in one or more lobes of one or both lungs.” Others have defined segmental PH more broadly as PH that does not follow a homogeneous distribution, with some parts of the pulmonary vasculature being exposed to higher pressures than others.3 This entity was included under the umbrella of World Heart Organization group 5 (PH caused by unclear or multifactorial mechanisms), because little is known about its pathophysiology and response to pulmonary arterial hypertension (PAH) therapies.1, 2 Segmental PH is most commonly encountered in patients with congenital heart disease (CHD) and carries notable similarities to PAH (Group 1.4.4, PAH associated with CHD) and group 4 of the PH classification (Group 4.2.4 PH in patients with congenital pulmonary artery [PA] stenoses), yet there is no systematic description of the broad spectrum of conditions encompassed by this entity or its distinct pathophysiological features and how these may affect management. We present herewith a consensus statement on segmental PH, including a working definition, range of conditions that may be classified under this entity, description of pathophysiology in terms of pulmonary vasculature, cardiovascular anatomy, and management principles.

Right-sided aortic arch with bilateral ductus: a rare case of nonconfluent pulmonary arteries without associated cardiac anomalies

Introduction Patent ductus arteriosus (PDA) accounts for 6 - 11% of all congenital heart defects. 1 ,2 Complications of PDA include congestive heart failure, repeated chest infections, pulmonary hypertension, and an increased risk of infective endocarditis. Transcatheter closure of PDA has largely replaced surgical ligation in different age groups. 3-7 Currently, surgical intervention is restri-cted to premature babies or small infants with large symptomatic PDA, cases with unfavorable duct ana-tomy, and whenever the cost of the closure devices is unaffordable. 1 PDA was the first example of congenital heart dis-ease to be treated by transcatheter closure, which beco-mes an established form of treatment for the majority of patients with PDA and as a safe alternative to sur-gery. The per-cutaneous technique was first described by Porstmanur et al ., 8 since then various devices such as Rashkind PDA umbrella, 9 button device, 10 PDA coils 11 and most recently the Amplatzer duct occluder (ADO) have been introduced. 12,13 The ADO device was designed to provide the most desirable characteri-stics for a percutaneous closure device that can be used in most if not all patients with PDA. These include user - friendly delivery system, high complete closure rate, small delivery system (allowing its use in small infants), trans-venous delivery route, ability to adapt to various PDA sizes and types, and the ability to retrieve or reposition the device prior to release from a secure delivery system. Common complications of trans-catheter closure of PDA include residual shunt, left pulmonary artery (LPA) obstruction, protrusion of the device into the aorta, and embolization of the device. 14-16 Incidence of complications increases with certain types and large size ducts, and with the use of multiple coils for occlu-sion. 17 There are only a few reports correlating out-come and complications with the learning curve and experience. 18-20 In this study, we are reporting our ini-tial experience with PDA closure using Amplatzer duct occluder (ADO). Our focus was on reporting the complications of trans-catheter closure of PDA using PDA closure devices. This study was carried out to evaluate the safety and efficacy of Amplatzer device for the transcatheter closure of PDA in our setup.