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Asian Journal of Surgery Jan 2022
Topics: Adult; Aortopulmonary Septal Defect; Coronary Vessel Anomalies; Humans
PubMed: 34801359
DOI: 10.1016/j.asjsur.2021.10.017 -
The Annals of Thoracic Surgery Jul 2021This study aimed to determine the factors related to reintervention, especially for pulmonary stenosis (PS), in patients with Taussig-Bing anomaly (TBA) after the...
BACKGROUND
This study aimed to determine the factors related to reintervention, especially for pulmonary stenosis (PS), in patients with Taussig-Bing anomaly (TBA) after the arterial switch operation.
METHODS
This retrospective study included 34 patients with TBA who underwent the arterial switch operation between 1993 and 2018. Preoperative anatomic and physiologic differences and long-term outcomes were determined using a case-matched control with transposition of the great arteries with ventricular septal defect and TBA with an anterior and rightward aorta.
RESULTS
At arterial switch operation, median age was 43 days (range, 16 to 102) and median body weight was 3.6 kg (range, 2.8 to 3.8 kg). Aortic arch obstruction and coronary anomalies were present in 64% and 41% of patients, respectively. The hospital mortality rate was 11%, including one cardiac death, and the late mortality rate was 2.9%. Furthermore, 41% patients underwent 26 reinterventions for PS. Patients undergoing PS-related reintervention had a significantly larger native pulmonary artery to aortic annulus size ratio than patients not receiving reintervention (1.69 vs 1.41, P = .02). This ratio was the only predictor of PS-related reintervention; it was significantly higher in the TBA group than in the transposition of great arteries/ventricular septal defect group. The PS-related reintervention was required more in the TBA group than in the transposition of great arteries/ventricular septal defect group.
CONCLUSIONS
Regardless of complex coronary anatomy and associated anomalies, early and late survival were acceptable. Postoperative PS was strongly associated with having a larger native pulmonary valve, suggesting that an optimal surgical reconstruction was required for achieving an appropriate aortopulmonary anatomic relationship during the arterial switch operation.
Topics: Arterial Switch Operation; Double Outlet Right Ventricle; Female; Humans; Infant; Infant, Newborn; Male; Proportional Hazards Models; Pulmonary Artery; Pulmonary Valve Stenosis; Reoperation; Retrospective Studies; Risk Factors
PubMed: 32795522
DOI: 10.1016/j.athoracsur.2020.06.016 -
Cureus Jul 2023The term "aortopulmonary window" (APW), often referred to as "aortopulmonary septal defect," refers to a rare congenital medical disorder where there is an improper...
The term "aortopulmonary window" (APW), often referred to as "aortopulmonary septal defect," refers to a rare congenital medical disorder where there is an improper direct link between the main pulmonary artery and the ascending aorta. It can be combined with other cardiac congenital conditions or be an isolated lesion. Herein, we report the incidental discovery of a minor, restrictive aortopulmonary septal defect in a 60-year-old male who denied having any clinical symptoms. Incidentally detected APW in adulthood is uncommon and, hence, can be readily overlooked, a fortiori, in asymptomatic patients.
PubMed: 37637538
DOI: 10.7759/cureus.42524 -
Annals of Pediatric Cardiology 2020Pulmonary atresia with the ventricular septal defect is a rare congenital heart defect with high anatomic variability. The most important management question relates to...
Comparison of computed tomography angiography versus cardiac catheterization for preoperative evaluation of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect.
INTRODUCTION
Pulmonary atresia with the ventricular septal defect is a rare congenital heart defect with high anatomic variability. The most important management question relates to the sources of pulmonary blood flow. The ability to differentiate between ductal dependence and major aortopulmonary collateral arteries is critical to achieving good outcomes and avoiding life-threatening hypoxia in the postneonatal period. Having accurate information about pulmonary arteries, major aortopulmonary collateral arteries, and sources of blood supply to each pulmonary segment is crucial for choosing the optimal surgical strategy. The purpose of this study is to compare computed tomography angiography (CTA) with cardiac catheterization for anatomic delineation of surgically relevant anatomy in pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries.
MATERIALS AND METHODS
Retrospective review of all children with pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries cared for at a large tertiary children's hospital who underwent cardiac catheterization with angiography and CTA close to each other without interval therapy. All studies were performed between 2007 and 2011.
RESULTS
There were 9 patients who met the inclusion criteria. Pulmonary artery anatomy (confluent vs. nonconfluent) was correctly identified in 9 patients by CTA and 8 patients by catheterization. There were no significant differences between CTA and catheterization in the identification of major aortopulmonary collateral arteries (mean = 3.4 collaterals/study via catheterization; mean = 3.1 collaterals/study via CTA; = 0.67). CTA was superior to catheterization in the delineation of segmental pulmonary blood flow ( = 0.006).
CONCLUSION
CTA and catheterization are equivalent in their ability to delineate pulmonary artery anatomy and major aortopulmonary collateral arteries.
PubMed: 32641882
DOI: 10.4103/apc.APC_94_19 -
Journal of Cardiology Cases Nov 2014Patients with polycythemia have an abnormally elevated hemoglobin, hematocrit, and red cell count. It is important to differentiate primary polycythemia from secondary...
Patients with polycythemia have an abnormally elevated hemoglobin, hematocrit, and red cell count. It is important to differentiate primary polycythemia from secondary causes since this can affect patient management and prognosis. We report the case of a young male, suspected to have primary polycythemia who was referred for a cardiology opinion after a bone marrow examination was normal and testing for Janus kinase gene mutation was negative. Presence of central cyanosis and clubbing, clinical evidence of severe pulmonary artery hypertension, and significant hypoxemia on arterial blood gas analysis suggested that the polycythemia was secondary to an intracardiac shunt. Transthoracic and contrast echocardiography revealed a large aortopulmonary window with right-to-left shunting. A 64-slice cardiac computed tomography imaging confirmed the diagnosis. In the developing world, it is not uncommon to encounter such unusual cases; careful attention to basic clinical signs and use of multimodality imaging are helpful in establishing the correct diagnosis. < Clinicians should be aware that it is important to distinguish between primary and secondary polycythemia. Although Eisenmenger syndrome is rare in the West, it is not uncommon to encounter such cases in the developing world. Since such patients are polycythemic, they often undergo unnecessary hematological investigations as happened in our case. Careful attention to basic clinical signs and a systematic approach with multimodality imaging were helpful in establishing the diagnosis.>.
PubMed: 30534241
DOI: 10.1016/j.jccase.2014.07.008 -
Archives of Gynecology and Obstetrics Dec 2021To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with truncus arteriosus communis (TAC) METHODS: All...
PURPOSE
To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with truncus arteriosus communis (TAC) METHODS: All cases of TAC diagnosed prenatally over a period of 8 years were retrospectively collected in two tertiary referral centers. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed.
RESULTS
Thirty nine cases of TAC were diagnosed prenatally. Mean gestational age at first diagnosis was 22 weeks (range 13-38). Two cases were lost follow-up. Correct prenatal diagnosis of TAC was made in 87.5% and of TAC subtype in 90.5%. Prenatal diagnosis was incorrect in three cases: one newborn had aortic atresia with ventricular septal defect (VSD) postnatally, one had hypo-plastic right ventricle with dextro transposition of the great arteries (d-TGA) with coarctation of the aorta and a third newborn had tetralogy of fallot (TOF) with abnormal origin of the left pulmonary artery arising from the ascending aorta postnatally. These 3 cases were excluded from further analysis. In 26.5% of cases, TAC was an isolated finding. 38.2% of fetuses had additional chromosomal anomalies. Among them, microdeletion 22q11.2 was most common with a prevalence of 17.6% in our cohort. Another 3 fetuses were highly suspicious for non-chromosomal genetic syndromes due to their additional extra-cardiac anomalies, but molecular diagnosis could not be provided. Major cardiac and extra-cardiac anomalies occurred in between 8.8% and 58.8%, respectively. Predominantly, extra-cardiac anomalies occurred in association with chromosomal anomalies. Additionally, severe IUGR occurred in 17.6%. There were 14 terminations of pregnancy (41.2%), 1 (2.9%) intrauterine fetal death, 5 postnatal deaths (14.7%) and 14 (41.2%) infants were alive at last follow-up. Intention-to-treat survival rate was 70%. Mean follow-up among survivors was 42 months (range 6-104). Postoperative health status among survivors was excellent in 78.6%, but 46.2% needed repeated re-interventions due to recurrent pulmonary artery or conduit stenosis. The other 21.4% of survivors were significantly impaired due to non-cardiac problems.
CONCLUSION
Truncus arteriosus communis is a rare and complex cardiac anomaly that can be diagnosed prenatally with high precision. TAC is frequently associated with chromosomal and extra-cardiac anomalies, leading to a high intrauterine and postnatal loss rate due to terminations and perioperative mortality. Without severe extra-cardiac anomalies, postoperative health status is excellent, independent of the subtype of TAC, but the prevalence of repeated interventions due to recurrent stenosis is high.
Topics: Female; Fetal Death; Fetus; Gestational Age; Heart Defects, Congenital; Humans; Infant; Infant, Newborn; Postoperative Complications; Pregnancy; Prenatal Diagnosis; Retrospective Studies; Thoracic Surgical Procedures; Ultrasonography, Prenatal
PubMed: 34028563
DOI: 10.1007/s00404-021-06067-x -
Journal of Thoracic Disease Mar 2020Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of...
BACKGROUND
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. Despite vast improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. The purpose of this chapter is to summarize the surgical algorithm used at Stanford University with a special focus on midline unifocalization.
METHODS
We have an experience with well over 300 patients undergoing primary surgical treatment of PA/VSD/MAPCAs. There were three pathways employed to accommodate the various forms of this heart defect. The dominant pathway is midline unifocalization, which was utilized in 80% of all patients. Less frequently employed strategies included creation of an aortopulmonary window (15%), and other approaches (5%).
RESULTS
For the patients who underwent a midline unifocalization, 85% had a single-stage complete repair while 15% had a unifocalization and shunt. The operative mortality was 1.5% for the single-stage complete repair group 8% for the unifocalization/shunt group. Ultimately, 97% of the midline unifocalization patients were repaired. However, the mean right ventricle to aortic pressure ratio was lower in patients who had a single-stage complete repair compared with an initial unifocalization/shunt (0.36±0.09 0.42±0.07).
CONCLUSIONS
The surgical treatment of PA/VSD/MAPCAs is made more complicated due to heterogeneity of MAPCA and pulmonary artery anatomy. The algorithm utilized at Stanford takes into account the anatomic heterogeneity and the results demonstrate that a high percentage of patients achieve full repair.
PubMed: 32274207
DOI: 10.21037/jtd.2019.10.44 -
Journal of Cardiothoracic Surgery Apr 2021This research aims to summarize the findings of the early single-stage revascularization of remnant pulmonary artery in unilateral absent intrapericardial pulmonary...
BACKGROUND
This research aims to summarize the findings of the early single-stage revascularization of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery.
METHODS
We retrospectively analyzed the medical records of 10 patients with unilateral absent pulmonary artery, in which 7 were right and 3 were left, the median age and mean weight at surgery was 4 months and 5.6 kg, respectively. The patients received operation from January 2009 to June 2020.
RESULTS
Ten patients, 1 case associated with atrial septal defect, 2 cases with tetralogy of Fallot, and 1 case with aortopulmonary window. The mean diameter of the affected hilar pulmonary artery remnants was 3.14 ± 1.09 mm (1.6-5 mm), and the Z value was - 3.66 ± 1.86 (range, - 6.7 to - 1.75). All the patients received single-stage revascularization: tube graft interposition in 3 patients, autologous pericardial roll in 4, direct anastomosis in one, and main pulmonary artery flap angioplasty in the rest 3. No hospital deaths occurred. Mean follow-up in this cohort was 3.3 ± 1.9 years One case underwent percutaneous balloon dilatation due to new pulmonary artery stenosis. Nonetheless, the results were encouraging, symptoms have improved in all patients. The median Z value of the latest ipsilateral pulmonary artery diameter was - 1.88 (range, - 4.52 to - 1.35), a significantly improvement when compared to the preoperative value. The Z value of that in patients who using Gore-Tex tube increased relatively small.
CONCLUSIONS
Single-stage pulmonary artery revascularization is effective at restoring normal antegrade flow to the affected lung, resulting in improved diameter of the PA, regression of pulmonary hypertension, and patient's symptoms. Revascularization by using the autologous tissue or autologous pericardium may obtain a preferred result. The new pulmonary artery stenosis certainly will need to be addressed in the long-term follow-up.
Topics: Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Pulmonary Artery; Retrospective Studies; Treatment Outcome; Vascular Malformations; Vascular Surgical Procedures
PubMed: 33849614
DOI: 10.1186/s13019-021-01481-3 -
Journal of Cardiac Surgery Apr 2022Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCAs) presents with variable anatomy with regard to the...
OBJECTIVES
Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCAs) presents with variable anatomy with regard to the pulmonary vasculature, requiring personalized surgical treatment. A protocol consisting of staged unifocalization and correction was employed.
METHODS
Since 1989, 39 consecutive patients were included (median age at first operation 13 months). In selected cases, a central aorto-pulmonary shunt was performed as the first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure, and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Echocardiographic data were obtained postoperatively and at interval follow-up.
RESULTS
In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, of which four have since died. One patient is awaiting further correction. A correction was performed successfully in 28 patients. Operative mortality was 3% and late mortality was 11%. Median follow-up after the correction was 19 years. Eleven patients required homograft replacement. Freedom from conduit replacement was 88%, 73%, and 60% at 5, 10, and 15 years respectively. Right ventricular function was reasonable or good in 75% of patients. All but one patient were in NYHA Class I or II.
CONCLUSIONS
After complete unifocalization 30/37 patients (81%) were considered correctable. The staged approach of PA, VSD, and SPCAs results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.
Topics: Collateral Circulation; Heart Defects, Congenital; Heart Septal Defects, Ventricular; Humans; Infant; Pulmonary Artery; Pulmonary Atresia; Retrospective Studies
PubMed: 35142386
DOI: 10.1111/jocs.16299 -
BMC Pediatrics Dec 2023Cantu syndrome is a rare and complex multisystem disorder characterized by hypertrichosis, facial dysmorphism, osteochondroplasia and cardiac abnormalities. With only... (Review)
Review
BACKGROUND
Cantu syndrome is a rare and complex multisystem disorder characterized by hypertrichosis, facial dysmorphism, osteochondroplasia and cardiac abnormalities. With only 150 cases reported worldwide, Cantu syndrome is now gaining wider recognition due to molecular testing and a growing body of literature that further characterizes the syndrome and some of its most important features. Cardiovascular pathology previously described in the literature include cardiomegaly, pericardial effusion, vascular dilation and tortuosity, and other congenital heart defects. However, cardiovascular involvement is highly variable amongst individuals with Cantu syndrome. In some instances, it can be extensive and severe requiring surgical management and long term follow up.
CASE PRESENTATION
Herein we report a case of a fourteen-year-old female who presented with worsening pericardial effusion of unknown etiology, and echocardiographic findings of concentric left ventricular hypertrophy, a mildly dilated aortic root and ascending aorta. Her medical history was notable for hemoptysis and an episode of pulmonary hemorrhage secondary to multiple aortopulmonary collaterals that were subsequently embolized in early childhood. She was initially managed with Ibuprofen and Colchicine but continued to worsen, and ultimately required a pericardial window for the management of refractory pericardial effusion. Imaging studies obtained on subsequent visits revealed multiple dilated and tortuous blood vessels in the head, neck, chest, and pelvis. A cardiomyopathy molecular studies panel was sent, and a pathogenic variant was identified in the ABCC9 gene, confirming the molecular diagnosis of autosomal dominant Cantu syndrome.
CONCLUSIONS
Vascular anomalies and significant cardiac involvement are often present in Cantu syndrome, however there are currently no established screening recommendations or surveillance protocols in place. The triad of hypertrichosis, facial dysmorphism, and unexplained cardiovascular involvement in any patient should raise suspicion for Cantu syndrome and warrant further investigation. Initial cardiac evaluation and follow up should be indicated in any patient with a clinical and/or molecular diagnosis of Cantu syndrome. Furthermore, whole body imaging should be utilized to evaluate the extent of vascular involvement and dictate long term monitoring and care.
Topics: Adolescent; Female; Humans; Cardiomegaly; Cardiovascular Abnormalities; Hypertrichosis; Osteochondrodysplasias; Pericardial Effusion; Vascular Malformations
PubMed: 38114927
DOI: 10.1186/s12887-023-04446-8