28-day-old Male with Non-Obstructing Supracardiac Total Anomalous Pulmonary Venous Connection and Atrial Septal Defect Presenting with Pulmonary Overcirculation

Publication ID Published Volume
9155 April 16, 2012 8


This simulation teaching case is intended to train the Emergency Medicine or Pediatric Resident or Fellow, or Critical Care Fellow, to rapidly assess and manage a critically ill infant with a congenital heart disease (CHD), in this case, Total Anomalous Pulmonary Venous Connection. Because undifferentiated clinical presentations of CHD are relatively uncommon and residents and fellows have less clinical experience to draw upon, it is important to offer a form of teaching that will allow the learner to engage the clinical process, potentially make mistakes that can be learned from without adverse affects on an actual patient, and utilize acquired skills to problem-solve in the absence of direct supervision.

Case participants are presented with an infant in respiratory distress and circulatory compromise with evidence of increased work of breathing, poor oxygen saturation, and central cyanosis. They will be expected to obtain an initial set of complete vital signs, perform a focused examination, place the infant on monitoring, obtain a history of present illness from the parent and stabilize the patient. They should articulate a differential diagnosis and establish a plan, but may initially proceed along multiple pathways (considering metabolic, infectious, cardiac, pulmonary, possibly endocrine and toxicologic etiologies). Treatment with prostaglandins for a ductal dependent etiology may be considered, but if employed will not result in clinical improvement. Participants should employ some variant of the hyperoxia test to help differentiate pulmonary from cardiac disease, as well as four extremity (or right upper and lower) blood pressures in consideration of coarctation of the aorta. Participants may respond to the poor initial oxygen saturation by administering oxygen by nasal canula or facemask, but this will result in either no change or a slight worsening of hypoxia (1-2 mm Hg) due to lowering pulmonary vascular resistance and increasing pulmonary overcirculation as might be expected in a case of Total Anomalous Pulmonary Venous Connection (TAPVC). The patient will deteriorate over the transition period, prompting endotracheal intubation that will improve the patient’s status due to positive pressure ventilation.

Participants will be expected to interpret the available data, including the electrocardiagram, laboratory results, chest radiograph, physical exam signs/symptoms, and hyperoxia test, and conclude that the infant has some form of congenital heart disease and is in a state of pulmonary overcirculation or congestive heart failure. They are not expected to specifically diagnose TAPVC. The significance of this particular variant, a non-obstructing supracardiac type with concurrent atrial septal defect will be discussed in the debriefing lecture. A treatment plan will be expected to address pulmonary edema, which may occur in conjunction with the consultation of a pediatric cardiologist. The case ends after endotracheal intubation, treatment with furosemide, and cardiology consultation are completed.

This pediatric simulation case offers learners the opportunity to manage an acute presentation of an infant with respiratory and circulatory instability secondary to an underlying congenital heart disease. It highlights clinical testing to distinguish between pulmonary and cardiac disease, and appropriate management steps to stabilize a critically ill infant in the team environment of a pediatric emergency critical care room. A presentation of total anomalous pulmonary venous connection (TAPVC) with atrial septal defect will be simulated, followed by debriefing with a lecture outlining the disease state and its evaluation and management.

Medical simulation is an effective training tool that allows learners to experience the evolution of a patient encounter and requires critical thinking and active participation. Although cases of TAPVC are uncommon, broader skills are reviewed in this patient encounter that include stabilizing airway, breathing, and circulation in a critically ill infant and recognizing the manifestation of a congenital heart disease and initiating emergency management.


Van Ness-Otunnu R, Kobayashi L, Ford S, Overly F. 28-day-old male with non-obstructing supracardiac total anomalous pulmonary venous connection and atrial septal defect presenting with pulmonary overcirculation. MedEdPORTAL Publications. 2012;8:9155. http://doi.org/10.15766/mep_2374-8265.9155

Contains time-sensitive information that will likely be inaccurate, obsolete, or irrelevant by April 16, 2015

Educational Objectives

  1. To assess airway, breathing, and circulation, identify key signs and symptoms such as retractions, grunting, cyanosis, and initiate stabilization with oxygenation, ventilation, and circulation support, when presented with an infant in respiratory distress.
  2. To initiate a treatment plan that may include positive pressure mechanical ventilation, diuretics, inotropic agents, and consultation of a pediatric cardiologist when presented with an infant in respiratory and circulatory distress with clinical, radiographic, and electrocardiographic findings suggestive of congenital heart disease and pulmonary edema.
  3. To obtain a complete medical history including history of present illness and review of systems, birth, pre-natal, maternal, feeding/stooling, family, social, and growth histories, and allergies, exposures, immunizations, medications, and surgery information.
  4. To auscultate for heart murmurs and lung sounds, employ hyperoxia testing to help differentiate between pulmonary and cardiac etiologies, and measure both upper and lower extremity blood pressures in consideration of coarctation of the aorta.
  5. To consult a pediatric cardiologist for expert opinion on more advanced management of pulmonary overcirculation, blood pressure control, and indications for inotropic support or afterload reduction as needed when presented with clinical data supporting a cardiac cause, after or while stabilizing the patient.


  • Total Anomalous Pulmonary Venous Connection, Pulmonary Overcirculation, Atrial Heart Septal Defects, Congenital Heart Disease

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ISSN 2374-8265