Successful Stent Implantation Into the Patent Ductus Arteriosus in Complex Cyanotic Congenital Heart Disease.

Birth-associated structural issues with the heart are known as congenital heart disorders or defects. They might alter the heart's regular blood flow. A 10-month-old female child presented to a tertiary care hospital with symptoms of recurrent cyanotic spells with episodes of desaturation a few months after birth. ECG findings depicted a normal sinus rhythm with a right axis deviation along the right ventricular forces. Two-dimensional echocardiography showed a tetralogy of Fallot with pulmonary atresia with a patent ductus arteriosus from the undersurface of the arch with confluent small pulmonary arteries. A coronary wire was passed through the left subclavian artery, and a 4 × 16 mm stent was deployed successfully. After the procedure, the patient's saturation improved, and she was extubated on the table. The patient was on heparin for 24 hours and was started on oral aspirin thereafter. This case was discharged on the third postoperative day and was advised to follow up.

Bilateral ductal stenting in an infant with pulmonary atresia and ventricular septal defect, non-confluent pulmonary arteries, and bilateral arterial duct.

Pulmonary atresia with ventricular septal defect, non-confluent pulmonary arteries, and bilateral arterial duct is a rare and complex CHD. Physiologic ductal closure may lead to life-threatening hypoxia. We present a case of successful bilateral ductal stenting as a bridge to further lower-risk surgical repair.

Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.

Tetralogy of Fallot with pulmonary atresia (ToF-PA) is a rare diagnosis that includes an extraordinarily heterogeneous group of complex anatomical findings with significant implications for physiology and prognosis. In addition to the classic findings of ToF, this particular diagnosis is characterized by complete failure of forward flow from the right ventricle to the pulmonary arterial system. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, most frequently via a patent ductus arteriosus, major aortopulmonary collaterals, or a combination of the two. The pathophysiology of ToF-PA is largely attributable to the abnormalities of the pulmonary vasculature. Ultimately, these patients require operative intervention to create a reliable, controlled source of pulmonary blood flow and ideally complete intracardiac repair. Even after operative correction, these patients remain at risk for pulmonary arterial stenoses and pulmonary hypertension. Although there have been significant advances in surgical and interventional management of ToF-PA leading to dramatic improvements in survival and long-term functional status, there is ongoing debate about the optimal management strategy given the risk of development of irreversible abnormalities of the pulmonary vasculature and the morbidity and mortality associated with sometimes multiple, complex operative interventions often occurring early in infancy. This review will discuss the findings in patients with ToF-PA with a focus on the perioperative and anesthetic management and will highlight challenges faced by the anesthesiologist in caring for these patients.

Correlating computed tomographic angiography of pulmonary circulation with clinical course and disease burden in patients with tetralogy of Fallot and pulmonary atresia.

PURPOSE: To determine the type of pulmonary circulation (PC) in patients with tetralogy of Fallot (TOF) and pulmonary atresia (TOF-PA) with the use of computed tomographic angiography (CTA), and describe their clinical courses, corrective surgery and disease burden. METHODS: 145 patients (median age 4 years, interquartile range: IQR2-8 years) were analyzed for PC which divided into 5 CTA-types and 11 subtypes based on presence of main pulmonary trunk (MPA) and confluent pulmonary arteries (confluence-PAs), presence of ductus arteriosus or major aortopulmonary collateral arteries (MAPCAs), respectively. Pulmonary arteries (PAs) were assessed by McGoon ratio and arborization. Corrective surgery or palliative management was recorded by type of PC. Disease burden was calculated as the sum of CTA, diagnostic angiography, and palliative management. RESULTS: The most common (N = 77, 53 %) PC was the presence of MPA with confluent-PAs (type-1) which was encountered mostly in TOF patients, followed by the presence of confluent-PAs with atretic MPA (type-2) (N = 47, 32 %) which found mainly in TOF-PA. McGoon ratio in type-1 (2.44 ± 0.84) was significantly larger than type-2 (1.61 ± 0.61) (median difference 0.84, 95 %CI 0.56-1.11, p < 0.001). Almost 2/3 of patients in type-1 (71 %) and 1/3 of patients in type-2 (34 %) achieved corrective surgery. There was no significant difference in amount of disease burden among the different PC, with the median value of 3 (IQR1-4). CONCLUSIONS: Types of PC allow suggestions for size and arborization of PAs and successful surgical correction with an inverse relationship with the numbers of MAPCAs. There is no significant difference in amount of disease burden among the types of PC.

Anaesthesia concerns and perioperative management in a child with DiGeorge syndrome with corrected tetralogy of Fallot with pulmonary atresia posted for laparoscopic orchidopexy: Case report.

DiGeorge syndrome is afflicted with multiple congenital anomalies such as conotruncal and craniofacial anomaly, immune system dysfunction and hypoplasia/aplasia of parathyroid glands. Laparoscopy is a preferred surgical approach over open orchidopexy due to better visualisation of impalpable testis avoiding long incision, minimal tissue damage and a faster recovery. We report a case of DiGeorge syndrome with corrected tetralogy of Fallot with pulmonary atresia in a 1-year-old male child posted for laparoscopic orchidopexy. The anaesthesiologists face unique challenges due to the multisystem involvement and the effects of laparoscopic surgery on multiple organs. Thorough understanding of DiGeorge syndrome is essential for a good perioperative outcome.

Aortic valve replacement for infective endocarditis in an adult with uncorrected tetralogy of Fallot and pulmonary atresia.

Infective endocarditis is one of the complications encountered in patients with uncorrected tetralogy of Fallot; however, there have been only limited reports on surgical treatment of this condition. A 38-year-old man with uncorrected tetralogy of Fallot with pulmonary atresia previously palliated with modified Blalock-Taussig shunt was diagnosed with aortic valve infective endocarditis and developed severe aortic regurgitation. He underwent urgent aortic valve replacement, during which intermittent hypothermic circulatory arrest was necessary to achieve adequate myocardial protection and bloodless surgical field due to massive arterial return into the left ventricle from the collateral pulmonary circulation.

Stenting the complex patent ductus arteriosus in tetralogy of Fallot with pulmonary atresia: challenges and outcomes.

Patent ductus arteriosus (PDA) stenting has gained acceptance for palliation in cyanotic congenital heart disease. The PDA in tetralogy of Fallot with pulmonary atresia (ToF-PA) arises, in the left aortic arch, from underneath the arch and connects to the proximal left pulmonary artery, often resulting in stenosis. The PDA is usually elongated and tortuous, making stent implantation challenging. Shorter duration of palliation, aggravation of branch pulmonary artery stenosis resulting in poor growth and difficulty at surgery makes ductal stenting controversial. Access via the carotid and axillary artery reduces complexity of the procedure and improves success, with recent data demonstrating good pulmonary artery growth. Advances in bioresorbable stents offer future promise and will likely resolve some controversies surrounding PDA stenting in ToF-PA.

Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot.

OBJECTIVES: Right ventricle to pulmonary artery connection (RVPA connection) without prosthetic material has been our ideal strategy to palliate pulmonary atresia with ventricular septal defect (VSD) or severe tetralogy of Fallot for the last decade. We speculate that RVPA connection ensures adequate postoperative haemodynamics for symptomatic neonates and promotes pulmonary artery rehabilitation. The present study was undertaken to assess the outcome of this strategy. METHODS: Between 2000 and 2010, among 107 patients who benefited from an RVPA connection, 57 were neonates. Forty-eight of these underwent autologous tissue reconstruction, 5 using left atrial appendage. Median weight was 2.9 kg (range 1.8-4.4). Median Nakata index was 100 mm2/m2 (range 17-185 mm2/m2); 12% had major aortopulmonary collaterals. All patients were reviewed retrospectively. End-points were death or complete repair; reintervention for restrictive pulmonary blood flow was considered as failure. At follow-up, we evaluated reintervention after complete repair, and quality of life. RESULTS: There were 2 early deaths (RV hypoplasia and RV failure) and 3 late sudden deaths (range 3-6 months). Pulmonary blood flow required to be increased in 8 patients: 4 underwent shunt after a median delay of 1 month; RVPA connection enlargement was needed in 3; 1 patient had percutaneous angioplasty. Finally, 47 patients (81%) had a complete repair, of which 70% were performed without prosthetic material at a median age of 7 months (range 2-53), with a median Nakata index of 221 mm2/m2 (range 102-891). One patient died early and 1 was a failure with opening of the VSD after intracardiac repair. At last follow-up, 4 patients were still awaiting repair, with 1 late death and 5 who had required reintervention after intracardiac repair; there were 3 conduit replacements and 2 balloon dilatation patch enlargements. CONCLUSIONS: The neonatal RVPA connection approach (i) provides an acceptable survival rate with a satisfactory haemodynamic adaptation, (ii) facilitates rehabilitation of PAs and (iii) avoids the use of prosthetic graft at correction.

Evaluation by MRA of aortic dilation late after repair of tetralogy of Fallot.

OBJECTIVES: This study evaluated predictors for aortic dilation (AD) in patients with repaired tetralogy of Fallot (rTOF) using magnetic resonance angiography (MRA). BACKGROUND: AD is common in patients with rTOF and may result in increased morbidity and mortality. There are no guidelines for evaluation of AD for rTOF patients. METHODS: All adults with rTOF who previously underwent MRA had retrospective aortic measurements at the sinuses of Valsalva (SoV) and ascending aorta (AsAo). Rate of change in diameter was determined in patients with multiple MRAs. Chart review identified risk factors for AD. Univariate and multivariate analyses tested predictors of AD. RESULTS: Of the 87 patients who met the inclusion criteria, 12 (14%) had AD. At baseline, mean diameter was 3.6 ± 0.6 cm and 3.1 ± 0.6 cm at the SoV and AsAo, respectively. The AsAo was larger than the SoV in 17%. Predictors of AD included male gender, age, right aortic arch, pregnancy, older age at complete repair, smoking, and systemic hypertension. Serial studies were available in 55 patients; the rate of growth was slow: 0.4 ± 0.9 mm/year (SoV) and 0.1 ± 0.8mm/year (AsAo). CONCLUSIONS: AD is common in rTOF at the SoV and AsAo. Transthoracic echocardiography, which does not always image the AsAo as well as MRA, may not image AD in rTOF in cases in which the AsAo is dilated. Although several risk factors correlate with AD in rTOF, the rate of aortic growth is slow, suggesting that rTOF patients may not require frequent aortic imaging.

Tetralogía de Fallot con atresia pulmonar: Morfopatología y anatomía quirúrgica / Tetralogy of Fallot with pulmonary atresia: Morphopathology and surgical anatomy

Se detalla la morfopatología de la tetralogía de Fallot con atresia pulmonar como un espectro de variaciones que sirve de fundamento para destacar la anatomía quirúrgica de esta cardiopatía y se muestra la base embriológica que determina su estructura. Se estudiaron 35 corazones con el sistema secuencial segmentario. Se determinó: situs atrial, conexiones entre los segmentos cardíacos y entre el ventrículo derecho y la vasculatura arterial pulmonar. El situs atrial fue solitus, predominó la conexión atrioventricular concordante (33), las conexiones ventriculoarteriales fueron concordantes (17), doble salida de ventrículo derecho (9) y única vía de salida (9). Todos presentaron atresia valvular pulmonar; el tronco pulmonar fue hipoplásico (20), atrésico proximal (6) y ausente (9), ramas pulmonares confluentes e hipoplásicas (25), ausencia de confluencia (5), ausencia total de ramas y de conductos arteriosos (5) y colaterales aortopulmonares (10). Los corazones mostraron un espectro morfopatológico de severidad que expresa la tendencia a la desaparición de la conexión entre el ventrículo derecho y la circulación arterial intrapulmonar. Se resaltan las formas de la irrigación arterial pulmonar como fundamento para unifocalizar el flujo hacia los pulmones y se hace hincapié en la utilidad de la clasificación anatomoquirúrgica de Barbero Marcial. El conocimiento embriológico es útil para entender las conexiones vasculares del ventrículo derecho con los derivados de los sextos arcos aórticos, los vasos arteriales intrapulmonares y las colaterales aortopulmonares.

The morphopathology of tetralogy of Fallot with pulmonary atresia is detailed as a spectrum of variations which is the foundation to highlight the surgical anatomy of this cardiopathy and it is shown the embryological basis which determines its structure. Thirty five hearts were studied with the methodology of the segmental sequential system. The atrial situs, the connections between the cardiac chambers and between the right ventricle and the arterial pulmonary vasculature were determined. The atrial situs was solitus, the concordant atrioventricular connection was the most frequent (33), the ventriculoarterial connections were concordant (17), double outlet right ventricle (9) and single outlet (9). All hearts had atresia of the pulmonary valve; the pulmonary trunk was hypoplastic (20), atretic proximally (6) and completely absent (9), confluent and hypoplastic pulmonary branches (25), absence of confluence (5), complete absence of pulmonary branches and arterial ducts (5) and presence of aortopulmonary collaterals (10). The hearts show a morphopathologic spectrum of severity which documents the tendency in disappearing the connection between the right ventricle and the intrapulmonary arterial circulation. The determination of the arterial supply to the lungs is highlighted to unifocalize the blood flow toward the lungs. The usefulness of Barbero Marcial's surgical classification is emphasized. The embryologic knowledge is basic in understanding the vascular connections between right ventricle and the derivatives of embryonic sixths aortic arches, the intrapulmonary arterial vessels and the aortopulmonary collaterals.