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Anomalous left coronary artery from the pulmonary artery is a rare congenital heart disease (CHD) with diverse clinical presentations despite the same anatomy. Factors determining this heterogeneous presentation are not well understood.
Method and Results:
We retrospectively investigated 14 patients (12 females) who underwent surgical repair of anomalous left coronary artery from the pulmonary artery. These patients were divided into three groups based upon the severity of initial presentation: (1) severe, life-threatening condition (n = 5), (2) mild-to-moderate distress (n = 6), and (3) asymptomatic (n = 3). All patients presented with left ventricular dilation and retrograde flow in left coronary artery by echocardiogram. Eight patients in (1) and (2) presented with severe left ventricular dysfunction. All but one showed abnormal ECG consistent with myocardial ischemia or infarction. Asymptomatic patients had preserved left ventricular systolic function despite ischemic findings on ECG. In 13 patients after surgical repair, all but one normalised left ventricular geometry and systolic function, suggesting nearly full myocardial recovery upon improvement of myocardial perfusion; 8 patients had residual echogenic papillary muscle with variable degree of mitral regurgitation.
Evidence of myocardial ischemic injury was present in all patients with anomalous left coronary artery from the pulmonary artery regardless of their initial presentation. Retrograde flow in left coronary artery, implying collateral vessel development from right coronary artery to left coronary artery, was noted in all patients, yet only few patients had preserved systolic function at the time of diagnosis. The balance between effective myocardial perfusion and a deleterious fistulous flow provided by these collaterals and the simultaneous haemodynamic status are what determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.
Coronary artery fistulas are relatively rare congenital or iatrogenic heart defects that can present with or without symptoms. Symptomatic patients manifest as myocardial ischaemia, arrhythmia, or heart failure. We present a asymptomatic child with a large left anterior descending coronary artery to right ventricular fistula.
Deep learning using convolutional neural networks represents a form of artificial intelligence where computers recognise patterns and make predictions based upon provided datasets. This study aimed to determine if a convolutional neural network could be trained to differentiate the location of the anterior ethmoidal artery as either adhered to the skull base or within a bone ‘mesentery’ on sinus computed tomography scans.
Coronal sinus computed tomography scans were reviewed by two otolaryngology residents for anterior ethmoidal artery location and used as data for the Google Inception-V3 convolutional neural network base. The classification layer of Inception-V3 was retrained in Python (programming language software) using a transfer learning method to interpret the computed tomography images.
A total of 675 images from 388 patients were used to train the convolutional neural network. A further 197 unique images were used to test the algorithm; this yielded a total accuracy of 82.7 per cent (95 per cent confidence interval = 77.7–87.8), kappa statistic of 0.62 and area under the curve of 0.86.
Convolutional neural networks demonstrate promise in identifying clinically important structures in functional endoscopic sinus surgery, such as anterior ethmoidal artery location on pre-operative sinus computed tomography.
Aortopulmonary window is a rare congenital heart lesion. It might be associated with other CHDs, as well as with anomalous origin of the coronary arteries. Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is the most commonly described coronary artery anomaly in association with aortopulmonary window. We are describing a premature neonate who was diagnosed to have aortopulmonary window and ARCAPA immediately after birth, and had a successful operation at the age of 4 months. This report highlights the importance of very careful assessment of the coronary arteries in patients with aortopulmonary window.
To improve the prenatal diagnosis for anomalous origin of pulmonary artery branches by comparing and analyzing different types of fetal echocardiography features.
Between June 2012 and December 2018, fetal echocardiographic features were analyzed retrospectively from fetuses with a prenatal diagnosis of anomalous origin of pulmonary artery branch. The main points of identification were summarized.
A total of 12 fetuses were diagnosed, including anomalous origin of a pulmonary artery branch from the innominate artery and six cases with unilateral absence of pulmonary artery. The shared characteristic sonographic finding was the lack of confluence at the bifurcation of the main pulmonary artery. The differences between the two conditions are highlighted by the origin of the anomalous vessel. In fetuses with anomalous origin of one pulmonary artery branch, the affected pulmonary artery arose from the posterior wall of the ascending aorta as noted on three vessels and trachea view as well as the long axis of the left ventricular outflow tract. This is in contrast to fetuses with unilateral absence of pulmonary artery, where the origin of affected pulmonary artery arises from the base of the innominate artery via the ipsilateral patent arterial duct as evident on the three vessels and trachea view and the coronal view of innominate (brachiocephalic) artery.
(1) The main similarity is an absence of a confluence at the bifurcation of the main pulmonary artery. (2) The main distinguishing feature is the origin of the anomalous vessel from either the subclavian or directly from the aorta.
Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs. The purpose of this study is to demonstrate, with this rare anomaly, the accurate place of the echocardiography to establish diagnosis especially in the systemic or supra-systemic pulmonary hypertension.
Stenting of ostial pulmonary artery stenosis presents several unique challenges. These include difficulty in defining anatomy and need for precise stent placement in order to avoid missing the ostial stenosis or jailing either the contralateral branch pulmonary artery or the ipsilateral upper lobe branch.
A retrospective review of outcomes was conducted in 1.5 or 2-ventricle patients who underwent stent placement for ostial branch pulmonary artery stenosis. Specific catheterisation lab techniques were reviewed.
Forty-seven branch pulmonary arteries underwent stent placement for ostial stenosis in 43 patients. The median age and weight were 3.7 (0.3–18.1) years and 14.2 (5.6–70.0) kg, respectively. Three (2–8) angiographic projections were needed to profile the ostial stenosis. Open-cell stents were used in 23 and stents were modified in 5 cases. Following stent implantation, the minimum diameter improved from 3.6 (0.8–10.5) to 8.1 (4.2–16.5) mm (p < 0.001). The gradient improved from 21 (0–66) to 4 (0–27) mmHg (p < 0.001). Stent malposition occurred in eight (17%) of the stents placed. Five migrated distally causing suboptimal ostial coverage necessitating placement of a second stent in four. Three migrated proximally and partially jailed the contralateral pulmonary artery. Intentional jailing of the upper lobe branch occurred in four additional cases. At a follow-up of 2.4 (0.3–4.9) years, 15 stents underwent further dilation and 1 had a second stent placed within the exiting stent.
Ostial branch pulmonary artery stenosis may require additional angiography to accurately define the ostial stenosis. Treatment with stents is effective but carries high rates of stent malposition.
This chapter reviews the basics of truncus arteriosus. The authors review the signs and symptoms as well as the anatomic classifications for truncus arteriosus. The associated congenital anomalies are presented in addition to the anesthetic concerns of caring for patients with truncus arteriosus defects.
Intercoronary communications are a very rare congenital coronary artery anomalies. We report a case of a man who underwent elective coronary angiography that showed a bidirectional direct intercoronary communication between right coronary and left circumflex arteries.
An 11-year-old male was presented with exertional chest pain and was diagnosed with atresia of the left main coronary artery. A stress nuclear perfusion imaging was negative at initial presentation, and a vasodilator stress cardiac MRI was again negative 5 years later. The patient has fully participated in competitive sports for 6 years with no occurrence of cardiac events.
Congenital right pulmonary artery to left atrium fistula is rare cause of cyanosis. It is an abnormal fistulous connection between right pulmonary artery and left atrium representing a direct communication between a pulmonary artery and vein with absence of capillary network connecting between these two. Cardiovascular examination usually remains normal. High index of suspicion on clinical examination and subsequent transthoracic echocardiography is needed to suspect this entity. Bubble contrast echocardiography usually confirms the diagnosis. Cardiac catheterization is used as diagnostic as well as therapeutic modality. Cardiac catheterization is useful in classifying the fistula and helps in transcatheter closure by embolization devices. Choice of devices depends on type of fistula, vascular access, and presence of atrial communication. Here, we are reporting 8-month-old girl presenting with type 2 right pulmonary artery to left atrium fistula, which underwent successful transcatheter closure by 6 mm/4 mm duct occluder (Heart R, Lifetech Scientific, Shenzhen, China). Early closure in this young age will prevent complications of cyanosis. The technical consideration, possible access, and closure techniques are discussed in this young infant.
A 65-year-old healthy woman presented with a 15-year history of binocular horizontal diplopia worse when looking left. She had previously been thoroughly investigated multiple times for a left sixth nerve palsy (6NP) 15 years ago and had three normal magnetic resonance imaging (MRI) scans of the brain/orbits with contrast, normal acetylcholine receptor antibodies, normal thyroid function tests, normal cerebrospinal fluid, and normal nerve conduction studies and single-fibre electromyography. She was treated with prism glasses, which resulted in resolution of her symptoms in primary position.
Abnormal small fistulous flows in the pulmonary artery were detected on routine transthoracic echocardiography in asymptomatic patients by colour Doppler echocardiography. The most likely diagnosis is small coronary artery-pulmonary artery fistulas. We evaluated the clinical, echocardiographic, and follow-up findings of 101 patients. The mean age at first echocardiographic evaluation was 4.3 ± 4.2 years. In 79 (78.2%) of the patients, fistula flow in the pulmonary artery was diagnosed at the first presentation and the remaining 22 patients (21.8%) were diagnosed between the 2nd and 10th examination. The echocardiography indication was cardiac murmur in 42 (41.6%), routine cardiac control in 30 (29.7%), additional CHD in 14 (13.8%), non-specific chest pain in 11 (10.9%), suspicion of inflammatory heart disease in 2 (2%), and syncope in 2 (2%) patients. In 70 (69.3%) patients, fistulous flow was located in the anterior aspect of the main pulmonary artery, in 23 (22.8%) patients on the aortic side of the pulmonary artery and in 8 (7.9%) patients on the right pulmonary artery. Additional cardiac anomalies were ventricular septal defect in 8, patent ductus arteriosus in 6, atrial septal defect in 5, mitral valve prolapse in 4, coarctation of aorta in 4, bicuspid aortic valve in 3, and Kawasaki syndrome in 1 patient. Sixty-four patients (63.3%) were followed during a mean of 52.6 ± 43.7 months. Spontaneous closure was detected in only three patients; the others remained almost unchanged during the follow-up. Since the fistulas are thin and hemodynamically insignificant, echocardiography is an appropriate method to monitor these patients without performing any invasive diagnostic procedures for the fistula source.
A 10-year-old female with heterotaxy-asplenia and complex CHD developed pulmonary arteriovenous malformations with associated cyanosis after Fontan completion. She underwent orthotopic heart transplantation, but her pulmonary arteriovenous malformations persisted with progressive worsening cyanosis. Elective transcatheter left pulmonary artery embolisation was performed 2 years post-transplant, which successfully normalised her oxygen saturation without a significant increase in pulmonary artery pressure.
Isolated subclavian artery is a rare anomaly. A second steal due to a patent arterial duct further reduces arm perfusion. Surgical anastomosis of the isolated vessel to aorta normalises arm perfusion. Simple echocardiographic clues aid in the diagnosis. An associated moderate sized ventricular septal defect was non-surgically closed along with catheter closure of the duct resulting in improved arm perfusion.
We report an extremely rare case of a 14-month-old girl who was diagnosed with a single right coronary artery with coronary artery fistula communicating with the right ventricle and congenital absence of left coronary artery. Angiography showed a dilated and tortuous single right coronary artery draining into the right ventricle, absence of left coronary system, and left ventricular coronary circulation supplied via collateral vessels.
This chapter examines the structure of the normal heart at the gross, microscopic and ultrastructural levels. The pericardium, myocardium, endocardium, valves, arteries, veins, lymphatics, nerves and conduction system are described in detail, together with common variants of normal. References are given to normal values for heart measurements throughout gestation, infancy and childhood.
This chapter is devoted to the macroscopic and microscopic appearance of myocardial ischaemia and includes discussion of regional myocardial infarction and of papillary muscle rupture. Coronary atherosclerosis can occur, albeit rarely, in the child, and this is discussed particularly in relation to hypercholesterolaemia. Antiphospholipid syndrome and haemolytic-uraemic syndrome are also discussed.