Transcatheter pulmonary valve replacement in surgically-created double-barrel right ventricular outflow tracts: A single center case-series.

This manuscript describes the feasibility and approach to the assessment and performance of transcatheter pulmonary valve replacement (tPVR) in patients with surgically-created "double-barrel" right ventricular outflow tracts (RVOT). Patients with tetralogy of Fallot may have coronary anomalies which prohibit the performance of traditional tetralogy of Fallot repair. In certain cases, this may necessitate the placement of a right ventricle to pulmonary artery conduit in addition to the native RVOT, which is left in situ, creating so-called "double-barrel" RVOTs. When these patients develop RVOT dysfunction later in life, they would typically be referred for reoperation due to concerns for risk of coronary compression associated with a transcatheter approach. However, whether a transcatheter approach with valve replacement in the native RVOT is feasible or safe is unknown. This was a retrospective review of patients with a surgically created "double-barrel" RVOTs who underwent cardiac catheterization for assessment of tPVR at Boston Children's Hospital. From July 2012 to July 2022, there were four patients with "double-barrel" RVOTs who underwent assessment for tPVR. The age at catheterization ranged between 22 and 39 years. In three out of four patients, coronary compression testing was negative. These three patients had successful tPVR in the native RVOT. At follow up, all three patients were free of greater than mild regurgitation by echocardiogram and had a maximum instantaneous gradient across the RVOT ranging between 20 and 33 mmHg. Performance of tPVR in patients with surgically created "double-barrel" RVOTs is feasible. The safety of this procedure depends crucially on coronary artery assessment at all stages.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA): Echocardiographic diagnosis in a critically ill newborn.

ARCAPA echocardiographic diagnosis. A network of small multiple coronary vessels, a systo-diastolic jet of flow into the pulmonary artery and a retrograde inverted «blue¼ flow into the RCA are the key signs. CT-scan confirms the diagnosis .

ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.

Unexpected and atypical clinical presentation of myocardial infarction in infants and children: complex pathogenesis of progressive and lethal disease.

BACKGROUND: Myocardial infarction is rare in children but frequently occurs unexpectedly with atypical presentation. It can cause a progressive lethal course unless prompt treatment is initiated. METHODS: Paediatric cases of myocardial infarction diagnosed by the presence of ischaemic myocardial insults and symptomatic ventricular dysfunction were reviewed retrospectively. RESULTS: Eighteen patients (5 days to 14 years of age; median 3 months) with myocardial infarction were studied. The aetiology was variable, including congenital coronary anomalies: anomalous left coronary artery from pulmonary artery (five patients), pulmonary atresia with intact ventricular septum with right ventricle-dependent coronary circulation (four), anomalous aortic origin of left coronary artery (three), and Williams syndrome with supravalvar aortic stenosis (one). Two acquired coronary anomalies occurred in one patient with undiagnosed Kawasaki disease with complete thrombotic obstruction of the left coronary artery and another with post-transplant cardiac allograft microangiopathy. Three patients developed thromboembolic coronary artery incidents in normal coronary anatomy. Fourteen patients were less than 1 year of age (78%). Electrocardiographic abnormalities were noted in all patients. Four patients required extracorporeal membrane oxygenation support for severe ventricular dysfunction, none of whom survived. Five patients underwent heart transplant. Five patients died during the same hospitalisation, and one patient died after discharge (overall mortality 33%). Transplant-free survival was 39%. CONCLUSION: Most myocardial infarction occurred in infants who presented with abrupt onset of non-specific clinical manifestations with progressively deteriorating haemodynamic status resulting in poor transplant-free survival rate. Early diagnosis and treatment are essential to prevent catastrophic outcomes.

Congenital dual anterior interventricular artery.

Dual anterior interventricular artery is a rare type of CHD. We reported a fifteen-year-old girl who underwent CT angiography that demonstrated one anterior interventricular artery from aorta and another from pulmonary artery.

Deep learning reconstruction for coronary CT angiography in patients with origin anomaly, stent or bypass graft.

PURPOSE: To develop and validate a deep learning (DL)-model for automatic reconstruction for coronary CT angiography (CCTA) in patients with origin anomaly, stent or bypass graft. MATERIAL AND METHODS: In this retrospective study, a DL model for automatic CCTA reconstruction was developed with training and validation sets from 6063 and 1962 patients. The algorithm was evaluated on an independent external test set of 812 patients (357 with origin anomaly or revascularization, 455 without). The image quality of DL reconstruction and manual reconstruction (using dedicated cardiac reconstruction software provided by CT vendors) was compared using a 5-point scale. The successful reconstruction rates and post-processing time for two methods were recorded. RESULTS: In the external test set, 812 patients (mean age, 64.0 ± 11.6, 100 with origin anomalies, 152 with stents, 105 with bypass grafts) were evaluated. The successful rates for automatic reconstruction were 100% (455/455), 97% (97/100), 100% (152/152), and 76.2% (80/105) in patients with native vessel, origin anomaly, stent, and bypass graft, respectively. The image quality scores were significantly higher for DL reconstruction than those for manual approach in all subgroups (4 vs. 3 for native vessel, 4 vs. 4 for origin anomaly, 4 vs. 3 for stent and 4 vs. 3 for bypass graft, all p < 0.001). The overall post-processing time was remarkably reduced for DL reconstruction compared to manual method (11 s vs. 465 s, p < 0.001). CONCLUSIONS: The developed DL model enabled accurate automatic CCTA reconstruction of bypass graft, stent and origin anomaly. It significantly reduced post-processing time and improved clinical workflow.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) in adult patients - a multimodality imaging approach.

Purpose: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital ano-maly of the origin of the coronary arteries. The prevalence of this anomaly in the adult patient population is low, and therefore there is virtually no original research on this topic. Reports are limited to case reports. Material and methods: We evaluated 16,264 computed tomography (CT) exams (cardiac and chest) performed in our heart imaging department between 2015 and 2022 on a dual-source 128-slice CT scanner (SOMATOM Definition Flash, Siemens Healthineers, Forchheim, Germany) and established a retrospective registry of adult patients (> 18 years old) with ALCAPA. The study included 7 cases. Next, we collected clinical and echocardiographic data, which could be assessed retrospectively. Results: We found 7 cases of ALCAPA in adult patients (0.043%). Three of them were female, and 4 were male. The age varied between 20 and 60 years. Echocardiographic findings, as well as the clinical course, varied widely. Conclusions: ALCAPA is an extremely rare anomaly, which nonetheless must be taken into clinical consideration. This lesion may be fatal during infancy. Data regarding adult patients is scarce. Multicentre registries are needed to establish a more detailed clinical profile of adults with this anomaly.

Acute coronary syndrome in an anomalous mid-LAD right coronary artery: Don't forget to look twice before turning left.

A 68-year-old female with past medical history of hypertension, hyperlipidemia, multiple sclerosis, diverticulitis, and tobacco use presented with 1 day of atypical chest pain after a recent diverticulitis flare. Initial workup was notable for a normal electrocardiogram but elevated high sensitivity troponin T (616 ng/L). Due to persistent symptoms, the patient was given antiplatelet therapy and taken urgently to the catheterization lab where she was found to have complete occlusion of an anomalous right coronary artery branching off the mid-left anterior descending artery. Angioplasty was performed with a drug-eluting stent and her symptoms resolved. The patient recovered well and was discharged on appropriate medical therapy. This case demonstrates a case of acute coronary syndrome in an extremely rare coronary congenital abnormality. Further research is needed on when to be suspicious for coronary anomalies on patients presenting with myocardial infarction.

Occlusion of the os of the left coronary artery by dysplastic aortic valve tissue presenting as progressive mitral insufficiency and cardiac arrest.

Mitral regurgitation in the neonatal period is relatively rare. It can be secondary to a congenital malformation of the valve apparatus or mitral valve dysfunction and deformation secondary to myocardial dysfunction or volume load of the left ventricle. Less commonly, it can be due to coronary artery abnormalities leading to mitral valve papillary muscle ischaemia and subsequent dysfunction. Such coronary artery abnormalities include anomalous left coronary artery from pulmonary artery, left main coronary artery atresia, or a thromboembolic phenomenon. In this study, we describe a newborn with a dysplastic aortic valve causing obstruction of the os of the left coronary artery leading to progressive mitral insufficiency.

Split right coronary artery and shepherd's crook course of direct origin of the conal artery: An unhitherto association in a case of rheumatic mitral stenosis.

In a 47-year-old lady, planned for redo percutaneous mitral commissurotomy for recurrent mitral valve stenosis, there was incidental detection of splitting of right coronary artery and direct origin and shepherd's crook course of the conal artery. Though these two anomalies have no hemodynamic significance, correct nomenclature and potential clinical implications have been described.

A rare unclassified coronary anomaly: Right coronary artery with dual ostia origin from the right aortic sinus of Valsalva.

BACKGROUND: The dual ostia of coronary artery is extremely rare entity. We present a case of old woman with right coronary artery with dual ostia origin from the right aortic sinus of Valsalva. METHODS AND RESULTS: A 52-year-old woman presented with discontinuous chest tightness and palpitation. she was referred to coronary computed tomography angiography (CCTA). The CCTA suggested an anomalous right coronary artery (RCA) origin from the right sinus of Valsalva (RSV) with dual ostia. DISCUSSION: The dual ostia of coronary anomaly is extremely rare entity. Though catheter angiography may be performed for preoperative identification of coronary anomalies, it is limited in the description of the relationship of the coronary arteries to the cardiac structures and great vessels. CCTA can help in noninvasive characterization of coronary anomalies with respect to their origin, course, and spatial relations. Interventional cardiologists and cardiac surgeons should be aware of the new coronary anomaly for appropriate procedural planning and intra-procedural management. CONCLUSION: The important imaging valueof CCTA can help in noninvasive characterization of coronary anomalies with respect to their origin, course, and spatial relations.

Rare case of anomalous origin of the left coronary artery from the pulmonary artery in a 4-year-old child.

Anomalous origin of the left coronary artery from the pulmonary artery is an exceedingly rare and potentially fatal congenital coronary anomaly that typically presents early in infancy. We report an unusual case of anomalous origin of the left coronary artery from the pulmonary artery in a 4-year-old child who presented later in life with vague respiratory and gastrointestinal symptoms and was found to have severe global cardiac dysfunction with evidence myocardial ischaemia.

Idiopathic Ventricular Fibrillation: Look for the Hidden Guilty-A case of aborted cardiac death.

We report a unique case of a 6-year-old male child with aborted sudden cardiac death due to ventricular fibrillation. A rare anomalous aortic origin of the right coronary artery was detected and supposed to be the cause of the malignant arrhythmia. Clinical exome sequencing did not reveal any pathogenic variant related to channelopathies nor other known heart-related genes. The patient underwent cardiac surgery and a cardiac defibrillator was implanted for secondary prevention.

Infantile dilated cardiomyopathy due to congenital left main coronary ostial stenosis.

Although a rare form of congenital heart disease, anomalies of the coronary arteries can present as heart failure in infants. The most common lesion is an anomalous left coronary artery arising from the pulmonary artery, but other abnormalities can present similarly. This case is an infant who is found to have left coronary ostial stenosis causing dilated cardiomyopathy.

Transcatheter occlusion of an anomalous origin of left coronary artery from pulmonary artery in an adult as an alternative to surgery.

Anomalous origin of the left coronary artery from the pulmonary artery causes heart failure and death in infancy. In rare adult survivors with well-developed collaterals, surgical left coronary ligation to arrest steal is often combined with bypass grafting. Transcatheter left coronary artery closure in a symptomatic adult as an alternative to surgical ligation resulted in complete resolution of inducible ischaemia on myocardial perfusion imaging.

An unusual anatomic variation of the Posterior Descending Coronary Artery.

PURPOSE: To report a rare coronary anatomic variation. METHODS: Review of the coronary angiogram of a patient with evidence of myocardial ischemia. RESULTS: The posterior descending artery was aberrant, originating from a large intermediate brunch. CONCLUSION: This anatomic variation has not been previously described.

Transcatheter closure of coronary artery fistula: A 21-year experience.

OBJECTIVES: To evaluate the outcomes of transcatheter coronary artery fistula (CAF) closure and to identify anatomic/procedural factors that may impact outcomes. BACKGROUND: Due to the rarity of CAF, reported experience with transcatheter closure remains limited and anatomic and procedural factors that may lead to unsuccessful closure, complications, or recanalization of CAF are unclear. METHODS: All patients who underwent transcatheter CAF closure at Mayo Clinic from 1997 to 2018 were retrospectively reviewed. CAF anatomic characteristics, procedural techniques, and clinical/angiographic outcomes were assessed. RESULTS: A total of 45 patients underwent transcatheter closure of 56 CAFs. The most commonly used devices were embolization coils in 40 (71.4%) CAFs, vascular occluders in 10 (17.8%), or covered stent in 2 (3.6%). Acute procedural success with no or trivial residual flow occurred in 50 (89.3%) CAFs. Residual flow was small in three (5.4%) and large in three (5.4%). Eight (17.8%) patients had complications, including device migration in three, intracranial hemorrhage from anticoagulation in one, and myocardial infarction (MI) in four. MI was a result of covered stent thrombosis or stagnation of flow after closure of large distal CAF. Twenty-two patients with 27 CAFs had follow-up angiography after successful index procedure at median time of 423 (IQ 97-1348) days. Of these, 23 (85.2%) had no/trace flow and 4 had large flow from recanalization. CONCLUSIONS: Transcatheter CAF closure is associated with a favorable acute procedural success and complication rate in selected patients. Procedural success and risk for complication are highly dependent on CAF anatomy and closure technique.

Technical success and long-term outcomes after anomalous right coronary artery stenting with cardiac computed tomography angiography correlation.

INTRODUCTION: Anomalous origin of coronary arteries has been observed in about 0.35-2.10% of the population. Patients with anomalous right coronary artery (ARCA) may present with significant symptoms, arrhythmias or ACS, and at times sudden death. Traditionally, surgical correction has been the recommended treatment. However, these may be technically challenging, and bypass grafting for such anomalies has the potential for graft failure because of competitive flow. We sought to determine the intermediate and long-term outcomes of drug-eluting stent placement for patients with symptomatic ARCA. We also looked at angiographic findings suggestive of interarterial course as confirmed by subsequent computed tomography (CT) findings. METHODS: Between January 2005 and December 2012, we enrolled 11 patients for elective percutaneous coronary intervention (PCI) of ARCA in a single center, prospective, nonrandomized fashion. Patients were followed up in clinic at 1 week, 3 months, 6 months, and 1 year, and then annually or more frequently if needed. All patients underwent a cardiac CT, as well as functional stress testing when needed to assess for recurrence of disease. RESULTS: All 11 of our patients, who presented with significant symptomatic stenosis with an ARCA, were successfully treated with PCI. Mean follow-up duration was 8.5 years. The only two deaths during follow-up were related to noncardiac causes (sepsis), with a mortality rate of 18.2%. Two patients had a positive functional study and on subsequent coronary angiography, one of them had significant in-stent restenosis (target lesion revascularization of 9.1%) and one distal to the stent (target vessel revascularization 9.1%). We found the observation of a "slit-like lesion" on angiography to have a sensitivity of 100% and specificity of 86% for the diagnosis of interarterial course of the anomalous vessel seen on subsequent CT. CONCLUSIONS: Our study results suggest that PCI of ARCA is an effective and low-risk alternative to surgical correction, with good procedural success and long-term outcomes. It can provide symptomatic relief in such patients and may reduce the risk of sudden death in younger patients, without the inherent risks associated with surgical repair.

Aberrant right coronary artery in a grown up congenital cardiac patient, successfully treated 46 years earlier with a double Starr-Edwards silastic ball valve replacement: a case report.

BACKGROUND: The Starr-Edwards ball valve prosthesis was successfully introduced in 1961-62 and largely used for aortic and mitral valve replacement. Even if Starr-Edwards valves have been widely replaced in clinical practice by other mechanical valves, they define a standard concerning long-term durability. CASE PRESENTATION: We describe the case of a 55-year-old man referred to our Department to perform a cardiac computed tomography (CCT), to better evaluate a severe dilation of ascending aorta discovered at echocardiography. The patient had been surgically treated 46 years earlier to correct a supra-cristal type ventricular septal defect. Both mitral and aortic valves were replaced, respectively due to bacterial mitral endocarditis and a fibrous sub-valvular aortic stenosis. In addition, the right coronary artery (RCA) was found to arise from the left coronary sinus. CONCLUSION: We report the longest lasting durability (46 years) of aortic and mitral Starr-Edwards valves successfully implanted in a patient simultaneously carrying a malignant anomalous origin of RCA.

Single coronary artery Left (SCA L)-Right coronary artery arising from mid-left anterior descending coronary artery: New variant of Lipton classification (SCA L-II) diagnosed by computed tomographic angiography.

Isolated single coronary artery (SCA) is a rare anomaly. Current classification of left and right is further classified based on the course of the anomalous vessel. We report two SCA L cases where right coronary artery (RCA) arose from mid-left anterior descending coronary artery (LAD). Our observation is a variation from the current Lipton classification SCA L Type II where RCA arose from left coronary artery before the LAD, in our cases the RCA arose from mid LAD after the first septal perforator. We believe that this variant should be described as SCA L Type II variant 2 (V2) while the original Lipton classification should be described as SCA L Type II variant 1 (V1).