Congenital Cardiac Anesthesia Society
A Section of the the Society for Pediatric Anesthesia

{“questions”:{“fghxj”:{“id”:”fghxj”,”mediaType”:”video”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2022\/09\/RVANGIO.mp4″,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Jared Spilka, MD and Michael A. Evans, MD \u2013 Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern Feinberg School of Medicine \r\n\r\nA 3-month-old presents to the cardiac catheterization laboratory for hemodynamic measurements and coronary angiography. Based on the angiograms illustrated below, what is the MOST LIKELY diagnosis (Image source, authors)? “,”desc”:””,”hint”:””},”a609q”:{“id”:”a609q”,”mediaType”:”video”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2022\/09\/LCAANGIO.mp4″,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:””,”desc”:”The Correct Answer is C. Ventriculo-coronary fistulae Pulmonary atresia with intact ventricular septum (PA\/IVS) is a congenital heart defect that occurs in about 0.6\/10,000 live births. PA\/IVS represents a large spectrum of anatomic variety. This anatomy varies with respect to the tricuspid valve development, degree of RV hypoplasia, infundibular development, and presence of coronary stenosis and\/or ventriculo-coronary fistulae. The development of the right ventricle and pulmonary valve ranges from membranous atresia of the pulmonary valve with mildly hypoplastic right ventricle (RV) and tricuspid valve (TV) and a well-developed infundibulum to a severely hypoplastic RV and TV, an underdeveloped infundibulum, and right-ventricular-dependent coronary circulation (RVDCC). As a result, the patent ductus arteriosus (PDA) is the sole source of pulmonary blood to the pulmonary arteries, which are typically confluent. Thus, PA\/IVS is considered single ventricle physiology at birth. An obligate right-to-left atrial level shunt allows for survival in-utero and after delivery. In addition, the tricuspid valve is abnormal, ranging from hypoplastic and severely stenotic to severely regurgitant. In some cases, the tricuspid valve is described as Ebsteinoid in character. \r\n\r\nA small tricuspid valve with a low Z-score and severe stenosis is associated with a severely hypertensive RV in which there is no egress of blood via the right ventricular outflow tract (RVOT). As a result, embryological coronary sinusoids act as a means of egress of blood while in utero and likely lead to the development of RV to coronary artery fistulae. The turbulent flow within these fistulae likely contribute to stenosis of the coronary arteries and at times interruptions of normal aorto-coronary blood flow leading to right ventricle dependent coronary circulation (RVDCC). In this circumstance, attempts to open the RVOT and decompress the right ventricle will lead to decreased perfusion of the ventricular wall, myocardial ischemia, and probable mortality. Hemodynamic cardiac catheterization with angiography is therefore critical to rule out the presence of RVDCC prior to any catheter-based or surgical intervention. \r\n\r\nThe demonstrated angiographic video shows the following: \r\n\u2022<\/strong>\tRight ventricular hand injection (upper right and left videos) \r\no\tHypoplastic, muscle-bound RV \r\no\tLack of blood flow through the pulmonary valve \r\no\tMultiple ventriculo-coronary fistulae to the proximal right main coronary artery and mid to distal left anterior descending (LAD) \r\no\tA ventriculo-coronary fistulous connection to a large collateral traveling leftward and filling the distal LAD \r\n\u2022<\/strong>\tSelective left coronary angiogram (lower right and left videos) \r\no\tLeft circumflex (LCx), left obtuse\/marginal (OM), and sinoatrial nodal branches seen in their entirety\r\no\tTruncated view of proximal LAD \r\no\t Lack of visualization of the mid to distal LAD \r\no\tComplete wash-in (reversal of flow) of the LAD\/diagonal and left main coronary artery \r\nIt can be deduced that this patient does not have RVDCC. In terms of the left coronary artery, there is dual blood supply from the RV through coronary fistulous connections and antegrade flow to the proximal LAD and LCx. The selective left coronary angiograms do not demonstrate coronary ostial atresia. However, a right selective coronary angiogram or an aortogram would be needed to demonstrate the patency of the right coronary ostia. Ultimately, this patient falls in a grey zone and could tolerate RV decompression. \r\n \r\nIn the presence of RVDCC and coronary ostial atresia\/stenoses, patients often require transplant evaluation and a systemic to pulmonary artery surgical shunt or PDA stent to maintain pulmonary blood flow and pressurization of the RV as a bridge. In some cases of PA\/IVS, the single ventricle pathway culminating in the Fontan palliation may be undertaken, but all stages tend to have significant risk of morbidity and mortality. In less-severe phenotypes of PA\/IVS, without the presence of RVDCC for example, it is reasonable to plan for a repair involving biventricular circulation. A simplified management algorithm can be seen below. \r\n\r\n \r\n\r\nAnomalous left (or right) coronary artery from the pulmonary artery (ALCAPA\/ARCAPA) is a defect in which one of the coronary arteries arises from the pulmonary artery resulting in myocardial perfusion with significantly desaturated blood. This eventually leads to myocardial ischemia and cardiomyopathy and requires surgical correction. Contrast injected into the pulmonary artery would reveal flow into the associated coronary artery in ALCAPA and ARCAPA. \r\n\r\nSingle coronary artery (SCA) is a defect in which the myocardium is perfused with a coronary artery that arises from a single coronary ostium. A left coronary artery may arise from the single right coronary or vice versa. This coronary pattern is noted with frequency in D-transposition of the great arteries, requires careful evaluation during repair, and can affect outcomes in D-TGA. Aortic root angiography would demonstrate contrast flow into a single ostium that then branches into right and left coronaries. \r\n\r\nCardiac allograft vasculopathy (CAV) is an accelerated form of coronary artery disease (CAD) that occurs due to concentric intimal hyperplasia (thickening) along the entire course of a coronary vessel. This pathology occurs after orthotopic heart transplantation and contributes significantly to mortality. The treatment for CAV is retransplantation. CAV is challenging to diagnose, as angiography is not as sensitive for the detection diffuse areas of narrowing that are seen in CAV. Focal areas of narrowing and plaques as seen in CAD are more easily detected by angiography. Many other imaging modalities are utilized to attempt early detection of CAV, which include vascular ultrasound, optical coherence tomography, computed tomography angiography, and cardiac MRI. \r\n\r\nREFERENCES: \r\nUngerleider R.M. et al. Critical Heart Disease in Infants and Children<\/em>. 3rd Edition. Elsevier. 2019. \r\n\r\nChikkabyrappa SM, Loomba RS, Tretter JT. Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management. Semin Cardiothorac Vasc Anesth<\/em>. 2018;22(3):245-255. doi:10.1177\/1089253218756757 \r\n\r\nDesmet W, Vanhaecke J, Vrolix M, et al. Isolated single coronary artery: a review of 50,000 consecutive coronary angiographies. Eur Heart J<\/em>. 1992;13(12):1637-1640. doi:10.1093\/oxfordjournals.eurheartj.a060117 \r\n\r\nPober JS, Chih S, Kobashigawa J, Madsen JC, Tellides G. Cardiac allograft vasculopathy: current review and future research directions. Cardiovasc Res<\/em>. 2021;117(13):2624-2638. doi:10.1093\/cvr\/cvab259 \r\n\r\nRickenbacher PR, Pinto FJ, Chenzbraun A, et al. Incidence and severity of transplant coronary artery disease early and up to 15 years after transplantation as detected by intravascular ultrasound. J Am Coll Cardiol<\/em>. 1995;25(1):171-177. doi:10.1016\/0735-1097(94)00323-i \r\n\r\nRamzy D, Rao V, Brahm J, Miriuka S, Delgado D, Ross HJ. Cardiac allograft vasculopathy: a review. Can J Surg<\/em>. 2005;48(4):319-327.\r\n\r\n\r\n\r\n\r\n”,”hint”:””,”answers”:{“hdei1”:{“id”:”hdei1″,”image”:””,”imageId”:””,”title”:”A. Anomalous Right Coronary Artery from the Pulmonary Artery (ARCAPA)\r\n”},”og9c2″:{“id”:”og9c2″,”image”:””,”imageId”:””,”title”:”B. Single Coronary Artery (SCA)”},”926mm”:{“id”:”926mm”,”image”:””,”imageId”:””,”title”:”C. Ventriculo-coronary fistulae”,”isCorrect”:”1″},”il21n”:{“id”:”il21n”,”image”:””,”imageId”:””,”title”:”D. Cardiac Allograft Vasculopathy (CAV)”}}}},”results”:{“qqsfo”:{“id”:”qqsfo”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:””,”redirect_url”:””}}}

{“questions”:{“vvqb5”:{“id”:”vvqb5″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Derik Neilson, DO – Benefis Health System, Great Falls, MT and\r\nDestiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nPhenylephrine has been reported to have mixed effects on cardiac output. From which vascular bed does the administration of phenylephrine contribute the MOST to an increase in cardiac output in hypovolemic patients? \r\n”,”desc”:””,”hint”:””,”answers”:{“ymzi6”:{“id”:”ymzi6″,”image”:””,”imageId”:””,”title”:”A.\tRenal”},”q7p7f”:{“id”:”q7p7f”,”image”:””,”imageId”:””,”title”:”B.\tAortic”},”mdmgs”:{“id”:”mdmgs”,”image”:””,”imageId”:””,”title”:”C.\tPulmonary”},”idjir”:{“id”:”idjir”,”image”:””,”imageId”:””,”title”:”D.\tSplanchnic”,”isCorrect”:”1″}}}},”results”:{“1a1t9”:{“id”:”1a1t9″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:””,”redirect_url”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2022\/09\/CCAS-QOW-Posted-9-1-2022.pdf”}}}

{“questions”:{“anj5q”:{“id”:”anj5q”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR and Meera Gangadharan, MD \u2013 McGovern Medical School, UTHealth, Houston \r\n\r\nA 17-year-old male adolescent with a history of hypoplastic left heart syndrome and Fontan palliation presents for diagnostic cardiac catheterization due to cyanosis with baseline saturations of 87% on room air. A transthoracic echocardiogram shows adequate right ventricular systolic function with mild tricuspid regurgitation and normal neo-aortic valve function. The hemodynamic catheterization reveals Fontan pressures of 13 mmHg, transpulmonary gradient of 4 mmHg, right ventricular end diastolic pressure of 9 mmHg, and an open Fontan pathway with device occlusion of the fenestration. A focused transthoracic echocardiography saline microbubble contrast study is performed with injections into the right pulmonary artery (PA) and produced the findings below. \r\nL Panel R Panel\r\nFigure 1. Apical 4 chamber view by transthoracic echocardiogram with saline contrast study. Before (L panel) and after (R panel) saline injection into the right pulmonary artery (RPA). (Image source credit: authors). \r\nWhat is the MOST LIKELY etiology of decreased oxygen saturation?\r\n\r\n”,”desc”:””,”hint”:””,”answers”:{“s0y26”:{“id”:”s0y26″,”image”:””,”imageId”:””,”title”:”A. Coronary sinus drainage”},”3btu7″:{“id”:”3btu7″,”image”:””,”imageId”:””,”title”:”B. Pulmonary arteriovenous malformations”,”isCorrect”:”1″},”au4hp”:{“id”:”au4hp”,”image”:””,”imageId”:””,”title”:”C. Elevated Fontan pressure”},”d20ha”:{“id”:”d20ha”,”image”:””,”imageId”:””,”title”:”D. Veno-venous collaterals”}}}},”results”:{“07lc1”:{“id”:”07lc1″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:””,”redirect_url”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2022\/08\/CCAS-QOW-Posted-8-25-2022.pdf”}}}

{“questions”:{“3isy2”:{“id”:”3isy2″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Jed Kinnick, MD and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR \r\n\r\nAccording to Van Praagh\u2019s embryological theory, Tetralogy of Fallot (TOF) is the result of a single anomaly which also gives rise to two other congenital cardiac anomalies. What is the single anomaly that gives the basis for Van Praagh\u2019s embryologic theory of TOF?”,”desc”:””,”hint”:””,”answers”:{“d4uh3”:{“id”:”d4uh3″,”image”:””,”imageId”:””,”title”:”A. Infundibular underdevelopment “,”isCorrect”:”1″},”8u5ss”:{“id”:”8u5ss”,”image”:””,”imageId”:””,”title”:”C. Leftward displacement of septum primum “},”0ectr”:{“id”:”0ectr”,”image”:””,”imageId”:””,”title”:”D. Abnormal communication between epicardial plexus and aortic buds”},”w5l5f”:{“id”:”w5l5f”,”image”:””,”imageId”:””,”title”:”E. Endocardial cushion defects \r\n\r\n”}}}},”results”:{“pz1pr”:{“id”:”pz1pr”,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:””,”redirect_url”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2022\/08\/CCAS-QOW-Posted-8-18-2022.pdf”}}}

{“questions”:{“entel”:{“id”:”entel”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Authors: Mikayla B. Troughton, MD \u2013 Northwestern University Feinberg School of Medicine, Chicago, IL and Destiny F. Chau, MD – Arkansas Children\u2019s Hospital\/University of Arkansas for Medical Sciences, Little Rock, AR. \r\n\r\nA previously healthy 10-year-old female child sustains a cardiac arrest while playing soccer. Return of spontaneous circulation was achieved with cardiopulmonary resuscitation. Upon hospital admission, an electrocardiogram demonstrated Q-waves and ST segment depression with inversion in the anterolateral leads. An echocardiogram revealed depressed left ventricular function, mild to moderate mitral valve regurgitation, lack of left ventricular outflow tract obstruction and the presence of abnormal collateral connections between the right and left coronary systems. A cardiac MRI was completed and demonstrated the following (Source credit: authors): \r\n\r\n\r\nFigure 1. Cardiac MRI image: Ao = Aorta; PA = Pulmonary Artery <\/em> \r\n\r\nWhich of the following is the MOST LIKELY etiology for the cardiac arrest? “,”desc”:””,”hint”:””,”answers”:{“u8uuh”:{“id”:”u8uuh”,”image”:””,”imageId”:””,”title”:”A.\tHypertrophic obstructive cardiomyopathy (HOCM) “},”e91ek”:{“id”:”e91ek”,”image”:””,”imageId”:””,”title”:”B.\tBrugada Syndrome “},”pbi87”:{“id”:”pbi87″,”image”:””,”imageId”:””,”title”:”C.\tAnomalous left coronary artery from the pulmonary artery (ALCAPA) “,”isCorrect”:”1″},”947sc”:{“id”:”947sc”,”image”:””,”imageId”:””,”title”:”D.\tHeritable dilated cardiomyopathy”}}}},”results”:{“k1yd2”:{“id”:”k1yd2″,”title”:””,”image”:””,”imageId”:””,”min”:”0″,”max”:”1″,”desc”:””,”redirect_url”:”https:\/\/ccasociety.org\/wp-content\/uploads\/2022\/08\/CCAS-QOW-Posted-8-11-2022.pdf”}}}