Major aortopulmonary collateral arteries in association with atrioventricular septal defect with balanced ventricles and trisomy 21.

A major aortopulmonary collateral artery is a rare and easily missed diagnosis that is usually associated with Tetralogy of Fallot or pulmonary atresia. We present two cases of major aortopulmonary collaterals associated with trisomy 21 and atrioventricular septal defect with balanced ventricles in which the diagnosis went undetected until after initial cardiac repair.

Aortopulmonary Collateral Arteries in Noncyanotic Congenital Heart Disease.

Development of major aortopulmonary collateral arteries are strongly associated with cyanotic congenital heart disease. However, they have rarely been reported in noncyanotic congenital heart disease. We report a rare case of a newborn originally diagnosed with an atrial septal defect, a ventricular septal defect, and pulmonary arterial hypertension who underwent complete repair. Failure to progress postoperatively lead to the delayed diagnosis of aortopulmonary collateral arteries. Percutaneous embolization and surgical ligation of aortopulmonary collateral arteries resulted in rapid recovery.

Stent implantation of an unusual morphology patent ductus arteriosus via Glidesheath slender.

The procedure of stenting the patent ductus arteriosus (PDA) is a palliative procedure applied as an alternative to surgery in newborns with ductus-dependent pulmonary circulation. However, it is still a very challenging method in patients with aortic arch anomalies. We describe our experience with a newborn with right atrial isomerism and dextrocardia, complete atrioventricular septal defect, aortic outlet right ventricle with pulmonary atresia, right aortic arch, and a PDA from the left innominate artery. Because the PDA was long and tortuous, we preferred placing three short stents instead of a single long stent. The procedure applied the femoral artery approach with a Glidesheath Slender to decrease arterial injuries. PDA stenting in challenging morphologies can be performed successfully using multiple short stents and via Glidesheath Slenders.

Atresia bronquial / Bronchial atresia

Introducción: La atresia bronquial es una patología poco frecuente, generalmente asintomática e incidental en las imágenes del tórax. La aproximación diagnóstica de esta entidad se puede hacer por radiografía y broncoscopia, donde se identifican algunos aspectos claves en el diagnóstico diferencial. De acuerdo las manifestaciones clínicas y posibles complicaciones el tratamiento puede ser desde conservador hasta quirúrgico con resección lobar o segmentaria. Objetivo: Presentar las características de un caso con atresia bronquial. Presentación de caso: Se presenta un caso de atresia bronquial en una paciente de 19 años de edad cuyo tratamiento definitivo fue bilobectomía media-inferior derecha. Conclusiones: La atresia bronquial es una entidad infrecuente que puede cursar de manera asintomática y ser detectada por un hallazgo radiológico en pacientes adultos de manera incidental. El diagnóstico se puede confirmar por broncoscopia y el tratamiento casi siempre es quirúrgico(AU)

Introduction: Bronchial atresia is a rare disease, generally asymptomatic and incidental in chest images. The diagnostic approach of this entity can be done by radiography and bronchoscopy, some key aspects are identified in the differential diagnosis. According to the clinical manifestations and possible complications, the treatment can range from conservative to surgical with lobar or segmental resection. Objective: To describe a case of bronchial atresia. Case report: A case of bronchial atresia is reported in a 19-year-old patient whose definitive treatment was a right-lower-middle bilobectomy. Conclusions: Bronchial atresia is a rare entity that can occur asymptomatically and be detected incidentally by a radiological finding in adult patients. The diagnosis can be confirmed by bronchoscopy, and treatment is almost always surgical(AU)

Bilateral coronary ostial atresia in a patient with pulmonary atresia and ventricular septal defect.

This is a case of a female infant with bilateral coronary ostial atresia associated with pulmonary atresia and ventricular septal defect. She developed coronary ischemia at 1-month of age, when she underwent an aortopulmonary shunt and an aorta-right ventricle shunt. The double-orifice tricuspid valve was separating the right ventricle from the left ventricle. She required extracorporeal cardiopulmonary support because of ventricular dysfunction and mitral regurgitation. Although she was temporarily weaned off the support after mitral valvuloplasty, she died from multiple organ failure. To the best of our knowledge, bilateral coronary ostial atresia associated with pulmonary atresia with ventricular septal defect has not been reported previously.

Safe Decompression of the Right Ventricle for PAIVS in Neonates With Coronary Fistulae: Including the Selective Use of Fistula Ligation to Avoid Coronary Steal.

BACKGROUND: There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary. METHODS: All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle. RESULTS: A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days (P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival. CONCLUSION: Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.

Balloon Valvuloplasty via the Pulmonary Artery Trunk for Treating Neonates With Severe Pulmonary Valve Disease.

BACKGROUND: Percutaneous balloon pulmonary valvuloplasty has proven to be a standard of care for neonates with severe pulmonary valve disease. However, the peripheral vessel injury, tricuspid chordae tendineae rupture, and cardiac tamponade could occur. Recently, we performed balloon valvuloplasty through pulmonary artery trunk. To date, the obtained outcome was promising. METHODS: Between January 2018 and December 2018, three neonates with critical pulmonary stenosis and two with membranous pulmonary atresia with intact ventricular septum were enrolled in our center. Balloon valvuloplasty through pulmonary artery trunk was performed in all patients. A 2-cm parasternal incision was made in the left third intercostal space. A guidewire was used to advance or perforate the pulmonary valve from the pulmonary artery trunk into the right ventricle, followed by balloon dilation of the valve. RESULTS: The procedure was successful in all patients. The oxygen saturation increased immediately after the balloon dilation, while the right ventricular systolic pressure and the gradient across the pulmonary valve decreased. No severe complications occurred. CONCLUSIONS: Balloon valvuloplasty through the pulmonary artery trunk is a safe and feasible alternative procedure. Thus, it could serve as a supplementary choice for treating severe pulmonary valve disease.

Survival With Respect to Morphology in Pulmonary Atresia and Intact Ventricular Septum in Sweden.

BACKGROUND: Patients born with pulmonary atresia and intact ventricular septum represent a challenge to pediatric cardiologists. Our objective was to study changes in survival with respect to morphology in all children born with pulmonary atresia and intact ventricular septum in Sweden during 36 years. METHODS: A retrospective, descriptive study based on medical reports and echocardiographic examinations consisting of those born between 1980 and 1998 (early group) and those born between 1999 and 2016 (late group). RESULTS: The cohort consists of 171 patients (early group, n = 86 and late group, n = 85) yielding an incidence of 4.35 and 4.46 per 100,000 live births, respectively. One-year survival in the early group was 76% compared to 92% in the late group (P = .0004). For patients with membranous atresia, one-year survival increased from 78% to 98%, and for muscular pulmonary atresia, from 68% to 85%. In patients with muscular pulmonary atresia and ventriculocoronary arterial communications, there was no significant increase in survival. Risk factors for death were being born in the early time period hazard ratio (HR), 6; 95% CI (2.33-14.28) P = .0002, low birth weight HR, 1.26; 95% CI (1.14-1.4) P < .0001 and having muscular pulmonary atresia HR, 3.74; 95% CI (1.71-8.19) P = .0010. CONCLUSION: The incidence of pulmonary atresia and intact ventricular septum remained unchanged during the study period. Survival has improved, especially for patients with membranous pulmonary atresia, while being born with muscular pulmonary atresia is still a risk factor for death. To further improve survival, greater focus on patients with muscular pulmonary atresia and ventriculocoronary arterial communications is required.

Three-Dimensional Printing, Virtual Reality and Mixed Reality for Pulmonary Atresia: Early Surgical Outcomes Evaluation.

BACKGROUND: Single-stage unifocalisation for pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCA) requires a high degree of three-dimensional (3D) anatomical imagination. A previous study has reported the application of a 3D-printed heart model with virtual reality (VR) or mixed reality (MR). However, few studies have evaluated the surgical outcomes of the 3D model with VR or MR in PA/VSD patients. METHODS: Three-dimensional (3D) heart models of five selected PA/VSD patients were derived from traditional imageology of their hearts. Using VR glasses, the 3D models were also visualised in the operating room. Both the 3D-printed heart models and preoperative evaluation by VR were used in the five selected patients for surgical simulation and better anatomical understanding. Mixed reality holograms were used as perioperative assistive tools. Surgical outcomes were assessed, including in-hospital and early follow-up clinical data. RESULTS: The use of these three new technologies had favourable feedback from the surgeons on intraoperative judgment. There were no in-hospital or early deaths. No reintervention was required until the last follow-up. Three (3) patients developed postoperative complications: one had right bundle branch block and ST-segment change, one had chest drainage >7 days (>40 mL/day) and one had pneumonia. CONCLUSION: The preoperative application of a 3D-printed heart model with VR or MR helped in aligning the surgical field. These technologies improved the understanding of complicated cardiac anatomy and achieved acceptable surgical outcomes as guiding surgical planning.

Rare cause of dynamic aortic obstruction in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries: a case report.

A 2-month-old infant with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries developed an aneurysmatic elongation of the tricuspid valve tissue that partially closed and dynamically protruded through the ventricular septal defect, beneath the aortic valve. This rare finding caused dynamic left ventricle outflow tract obstruction and recurrent cardiac arrests and ultimately required surgical intervention.

Segmental Pulmonary Hypertension in Children with Congenital Heart Disease.

Segmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to confirm and assess pulmonary vascular disease in subjects with major aorto-pulmonary collaterals. We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies.

Percutaneous Closure of a "Doughnut Leak" Around an Atrial Septal Occluder Device in a Patient With Recurrent Migraines.

A 10-year-old girl with pulmonary atresia status post transannular patch repair and secundum atrial septal defect (ASD) underwent percutaneous ASD closure 5 years earlier. There was improvement in arterial saturation from 86% to 98% with minimal residual right-to-left shunt. Despite subsequent medical therapy, she developed recurrent migraines, which led us to pursue percutaneous closure of the shunt.

Unusual Case of Common Arterial Trunk With Atresia of the Right Pulmonary Artery and Aortopulmonary Collaterals.

We describe a two-year-old African girl with late diagnosis of unusual case of common arterial trunk with two separate pulmonary artery branch origins from the ascending aorta, hypoplastic right pulmonary artery that becomes atretic and reconstitutes at hilum, and three aortopulmonary collaterals providing right lung blood supply. She underwent single-stage intracardiac repair and unifocalization of collaterals.

A case of COVID-19 in a patient with a univentricular heart post total cavopulmonary connection (Fontan) surgery.

Coronavirus disease 2019 (COVID-19) has caused a global pandemic which has affected patients and healthcare systems around the world. Patients with underlying health conditions seem to be more severely affected. There are limited reports of patients with univentricular circulations and COVID 19; thus, we report a case of COVID-19 in a patient with a univentricular circulation.

Transcatheter Approach for Critical Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum in Young Infants Using the Simmons Catheter.

METHODS AND RESULTS: We retrospectively reviewed 52 young infants, 41 of whom had CPS and 11 had PA/IVS, in a single center from June 2009 to October 2017. Patients were divided into three groups according to the type of catheter used to enter through the RVOT. The unique structure of the Simmons catheter allowed it to be maneuvered directly into the RVOT within a few minutes. Compared with the other two groups, the Simmons catheter group had a significantly shorter fluoroscopy time entering through the RVOT (P < 0.001) and a shorter total X-ray exposure time (P < 0.001). Furthermore, compared with the floating catheter group, the success rate of surgery was much higher in the Simmons catheter group (P < 0.001). CONCLUSIONS: The Simmons catheter is a safe and effective method to enter through the RVOT in infants with CPS or PA/IVS. Therefore, the Simmons catheter could be an alternative catheter when entering through the RVOT in young infants, especially neonates with low birth weight.

Ventricular Septal Defect Pulmonary Atresia: Provisional Bifurcation Strategy for Confluence Stenosis During Patent Ductus Arteriosis Stenting.

Neonates with pulmonary atresia survive only if duct patency is maintained before staged surgical repair. Ductal stenting is an effective alternative to conventional shunt surgery, as it avoids thoracotomy. With today's generation of coronary stents having better profile, flexibility, and trackability, it may be achieved safely, with less difficulty than previously described. The strategy during patent ductus arteriosus (PDA) stenting with trifurcation narrowing is a real challenge. We present an underweight newborn with ventricular septal defect pulmonary atresia, restrictive PDA, and bilateral branch stenosis at the ostia. We performed a successful PDA stenting as well as addressed the ostia of branch pulmonary arteries.

Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

OBJECTIVES: Critical pulmonary stenosis (PS) and pulmonary atresia with intact ventricular septum (PAIVS) require urgent neonatal intervention. Since PS may be more insidious than PAIVS during gestation, we hypothesized that neonates with PS would have lower rates of prenatal detection than PAIVS. METHODS: We performed a retrospective chart review of all neonates who underwent diagnostic or interventional cardiac catheterization between 2000 and 2014 for critical PS or PAIVS. The rates of prenatal diagnosis were calculated for PS and PAIVS. Prenatal and postnatal echocardiographic data were reviewed. RESULTS: 178 patients met inclusion criteria: 91 with critical PS and 87 with PAIVS. The prenatal diagnosis rate for critical PS was lower than for PAIVS at 37% (34/91) vs 60% (52/87) (P = .003). At the time of diagnosis at a median gestational age of 25 weeks, the median TV z-score for patients with critical PS was larger than in PAIVS (-0.15 vs -3.0 P = .004). CONCLUSION: Critical PS had a lower prenatal detection rate than PAIVS, likely due to a relatively normal 4-chamber view at the time of routine second trimester screening in patients with PS. Color flow Doppler of the outflow tracts may improve detection, since outflow tracts may appear normal by 2D imaging.

Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum.

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one such case which was diagnosed prenatally to have tetralogy of Fallot with absent pulmonary valve and identified postnatally to have nonconfluent pulmonary artery branches in addition. We discuss the conundrum of respiratory management in this patient pre- and postoperatively due to a unique ventilation perfusion mismatch problem, which varies between the two lungs.

Right Ventricle-Dependent Coronary Circulation: Location of Obstruction Is Associated With Survival.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PAIVS) with right ventricle-dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium. METHODS: We conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995 to 2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments, using the SYNTAX score criteria. Transplant-free survival was compared between patients with proximal and distal obstruction, then these groups were compared with patients without RVDCC. RESULTS: Of 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for patients with RVDCC was 1.8 years (interquartile range, 0.3-8.1 years). All deaths (10 of 28, 36%) occurred at 6 months old or earlier. Proximal coronary artery obstruction was associated with decreased transplant-free survival relative to distal obstruction (hazard ratio = 3.63; 95% confidence interval, 1.01-13.00; P = .048). Transplant-free survival at 1 year was 33% and 70% in the proximal and distal obstruction groups, respectively. Compared with patients without RVDCC, patients with proximal obstruction had significantly lower transplant-free survival (P < .001), whereas patients with distal obstruction did not (P = .217). CONCLUSIONS: The location of coronary artery obstruction affects clinical outcome and may represent a potential branch point in the management for PAIVS with RVDCC.