Coarctation and interrupted aortic arch — structured clinical encounter

Structured encounter testing the approach to a three-day-old neonate who was feeding well then collapses with shock, metabolic acidosis, weak femoral pulses and differential cyanosis: the prostaglandin-E1-first resuscitation rule, the echocardiographic confirmation, the ductal-dependent mechanism, the syndromic work-up, and the conversation with the family about the transfer to a cardiac centre.

structured clinical encounter

Prompt

A three-day-old term male infant, born vaginally at 39 weeks after an uncomplicated pregnancy, was feeding and thriving until this morning. His mother reports poor feeding, grunting and cool, mottled skin. In the emergency department he is shocked: heart rate 185, respiratory rate 72, capillary refill five seconds, and central cyanosis that clears with oxygen. The brachial pulses are easily palpable and bounding, but the femoral pulses are weak and delayed. The pre-ductal saturation is 96% and the post-ductal saturation is 70%. The blood gas shows a pH of 7.08, a base excess of minus fourteen and a lactate of 9 mmol per litre. A ductal-dependent arch obstruction is the working diagnosis. You are the paediatric registrar working through the recognition, the immediate resuscitation, the confirmatory investigation and the family conversation.

Encounter structure

The candidate works through the case in five phases: [4]

Recognition (5 minutes): Identify the ductal-dependent cardiac picture from the weak femoral pulses and the differential cyanosis; distinguish it from septic shock by the pulse pattern; state the working diagnosis of coarctation or interrupted aortic arch. [3]
Immediate resuscitation (5 minutes): Start prostaglandin E1 at 0.01 to 0.05 micrograms per kilogram per minute before the echocardiogram; anticipate apnoea and prepare to intubate; correct the metabolic acidosis with fluid and inotropes guided by perfusion and lactate. [4]
Investigation and stabilisation (5 minutes): Confirm the anatomy on echocardiography (site of obstruction, arch morphology, VSD, bicuspid valve, ductal patency); check the calcium for 22q11.2 hypocalcaemia; arrange transfer to a cardiac centre with the baby intubated. [7]
The family conversation (5 minutes): Explain the ductal-dependent mechanism in plain language; describe the immediate plan (PGE1, transfer, surgery); acknowledge the fear while giving honest reassurance about modern surgical outcomes. [2]
Follow-up planning (5 minutes): Outline the lifelong surveillance for re-coarctation, residual hypertension, aortic vasculopathy and bicuspid-valve disease; describe the transition to adult congenital heart disease services. [11]

References

[1]Salciccioli KB; Zachariah JP Coarctation of the Aorta: Modern Paradigms Across the Lifespan. Hypertension, 2023.PMID 37476999
[2]Kim YY; Andrade L; Cook SC Aortic Coarctation. Cardiol Clin, 2020.PMID 32622489
[3]Hede SV; DeVore G; Satou G; et al Neonatal management of prenatally suspected coarctation of the aorta. Prenat Diagn, 2020.PMID 32277716
[4]Bansal N; Balakrishnan PL; Aggarwal S Prostaglandin Infusion in Neonate With Severe Coarctation of the Aorta With Closed Ductus Arteriosus — A Case Report and Review of the Literature. World J Pediatr Congenit Heart Surg, 2020.PMID 31010402
[5]LaPar DJ; Baird CW Surgical Considerations in Interrupted Aortic Arch. Semin Cardiothorac Vasc Anesth, 2018.PMID 29774793
[6]Schreiber C; Mazzitelli D; Haehnel JC; et al The interrupted aortic arch: an overview after 20 years of surgical treatment. Eur J Cardiothorac Surg, 1997.PMID 9332928
[7]Burbano-Vera N; Zaleski KL; Latham GJ; et al Perioperative and Anesthetic Considerations in Interrupted Aortic Arch. Semin Cardiothorac Vasc Anesth, 2018.PMID 29742969
[8]Ron HA; Crowley TB; Liu Y; et al Improved Outcomes in Patients with 22q11.2 Deletion Syndrome and Diagnosis of Interrupted Aortic Arch Prior to Birth Hospital Discharge, a Retrospective Study. Genes (Basel), 2022.PMID 36672801
[9]Eckhauser A; South ST; Meyers L; et al Turner Syndrome in Girls Presenting with Coarctation of the Aorta. J Pediatr, 2015.PMID 26323199
[10]Wu Y; Jin X; Kuang H; et al Is balloon angioplasty superior to surgery in the treatment of paediatric native coarctation of the aorta: a systematic review and meta-analysis. Interact Cardiovasc Thorac Surg, 2019.PMID 30060099
[11]Toro-Salazar OH; Steinberger J; Thomas W; et al Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol, 2002.PMID 11867038
[12]Sinning C; Zengin E; Kozlik-Feldmann R; et al Bicuspid aortic valve and aortic coarctation in congenital heart disease — important aspects for treatment with focus on aortic vasculopathy. Cardiovasc Diagn Ther, 2018.PMID 30740325

Coarctation and interrupted aortic arch — structured clinical encounter

structured clinical encounter

References

[1]Salciccioli KB; Zachariah JP Coarctation of the Aorta: Modern Paradigms Across the Lifespan. Hypertension, 2023.PMID 37476999

[2]Kim YY; Andrade L; Cook SC Aortic Coarctation. Cardiol Clin, 2020.PMID 32622489

[3]Hede SV; DeVore G; Satou G; et al Neonatal management of prenatally suspected coarctation of the aorta. Prenat Diagn, 2020.PMID 32277716

[4]Bansal N; Balakrishnan PL; Aggarwal S Prostaglandin Infusion in Neonate With Severe Coarctation of the Aorta With Closed Ductus Arteriosus — A Case Report and Review of the Literature. World J Pediatr Congenit Heart Surg, 2020.PMID 31010402

[5]LaPar DJ; Baird CW Surgical Considerations in Interrupted Aortic Arch. Semin Cardiothorac Vasc Anesth, 2018.PMID 29774793

[6]Schreiber C; Mazzitelli D; Haehnel JC; et al The interrupted aortic arch: an overview after 20 years of surgical treatment. Eur J Cardiothorac Surg, 1997.PMID 9332928

[7]Burbano-Vera N; Zaleski KL; Latham GJ; et al Perioperative and Anesthetic Considerations in Interrupted Aortic Arch. Semin Cardiothorac Vasc Anesth, 2018.PMID 29742969

[8]Ron HA; Crowley TB; Liu Y; et al Improved Outcomes in Patients with 22q11.2 Deletion Syndrome and Diagnosis of Interrupted Aortic Arch Prior to Birth Hospital Discharge, a Retrospective Study. Genes (Basel), 2022.PMID 36672801

[9]Eckhauser A; South ST; Meyers L; et al Turner Syndrome in Girls Presenting with Coarctation of the Aorta. J Pediatr, 2015.PMID 26323199

[10]Wu Y; Jin X; Kuang H; et al Is balloon angioplasty superior to surgery in the treatment of paediatric native coarctation of the aorta: a systematic review and meta-analysis. Interact Cardiovasc Thorac Surg, 2019.PMID 30060099

[11]Toro-Salazar OH; Steinberger J; Thomas W; et al Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol, 2002.PMID 11867038

[12]Sinning C; Zengin E; Kozlik-Feldmann R; et al Bicuspid aortic valve and aortic coarctation in congenital heart disease — important aspects for treatment with focus on aortic vasculopathy. Cardiovasc Diagn Ther, 2018.PMID 30740325