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 A 29 year-old female who had an anomalous left main coronary
artery from the pulmonary artery (ALCAPA) and subsequently
underwent the Takeuchi procedure early in life presented for
evaluation of palpitations. She had worsening of her pulmonic
stenosis on a recent echocardiogram with her right and left
ventricular function being preserved. Her holter monitor showed
non-sustained ventricular tachycardia (NSVT) that correlated with
her palpations. Cardiac MRI was ordered to assess for any left
ventricular scar that could account for her NSVT and to assess
left main coronary anatomy before considering coronary
angiogram and an invasive hemodynamic study.
 Incidence is about 1 in 300,000 live births1
 0.25-0.5% of children with congenital heart disease1
 90% mortality in the first year if not treated1,2
 Definitive treatment – surgery1,2
› Direct implantation of left main into the aorta
 Most common now
› Coronary artery bypass grafting
› Takeuchi procedure
 Creating a baffle from the native pulmonary artery to connect the aorta to
the left main artery
1. Secinaro A, Ntsinjana H, et al. Cardiovascular magnetic resonance findings in repaired anomalous left coronary artery to
pulmonary artery connection (ALCAPA). Journal of Cardiovascular Magnetic Resonance. 2011. 13:27
2. Ginde S, Earing M, et al. Late Complications After Takeuchi Repair of Anomalous Left Coronary Artery From the
Pulmonary Artery: Case Series and Review of Literature. Pediatric Cardiology. 2012. 33; 1115-23.
 CMR helps with delineating anatomy
› Using 3-D Heart Navigation
 CMR to evaluate LV Scar
› Characteristically, patients who ALCAPA have sub-endocardial late
gadolinium enhancement (LGE) of the basal anterolateral wall and
sometimes papillary muscle1
 CMR
› Extremely valuable tool in defining coronary anatomy in adult patients
who have undergone surgery as young children for congenital
abnormalities
› May obviate the need for invasive procedures
1. Secinaro A, Ntsinjana H, et al. Cardiovascular magnetic resonance findings in repaired anomalous left coronary
artery to pulmonary artery connection (ALCAPA). Journal of Cardiovascular Magnetic Resonance. 2011. 13:27
 Gyanendra Sharma, M.D. – Director of Echocardiogram
Laboratory, Professor of Medicine, Division of Cardiology
 Jayanth H. Keshavamurthy, M.D. – Assistant Professor of
Radiology
ALCAPA AND TAKEUCHI PROCEDURE

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ALCAPA AND TAKEUCHI PROCEDURE

  • 1.
  • 2.  A 29 year-old female who had an anomalous left main coronary artery from the pulmonary artery (ALCAPA) and subsequently underwent the Takeuchi procedure early in life presented for evaluation of palpitations. She had worsening of her pulmonic stenosis on a recent echocardiogram with her right and left ventricular function being preserved. Her holter monitor showed non-sustained ventricular tachycardia (NSVT) that correlated with her palpations. Cardiac MRI was ordered to assess for any left ventricular scar that could account for her NSVT and to assess left main coronary anatomy before considering coronary angiogram and an invasive hemodynamic study.
  • 3.  Incidence is about 1 in 300,000 live births1  0.25-0.5% of children with congenital heart disease1  90% mortality in the first year if not treated1,2  Definitive treatment – surgery1,2 › Direct implantation of left main into the aorta  Most common now › Coronary artery bypass grafting › Takeuchi procedure  Creating a baffle from the native pulmonary artery to connect the aorta to the left main artery 1. Secinaro A, Ntsinjana H, et al. Cardiovascular magnetic resonance findings in repaired anomalous left coronary artery to pulmonary artery connection (ALCAPA). Journal of Cardiovascular Magnetic Resonance. 2011. 13:27 2. Ginde S, Earing M, et al. Late Complications After Takeuchi Repair of Anomalous Left Coronary Artery From the Pulmonary Artery: Case Series and Review of Literature. Pediatric Cardiology. 2012. 33; 1115-23.
  • 4.
  • 5.
  • 6.
  • 7.
  • 8.
  • 9.  CMR helps with delineating anatomy › Using 3-D Heart Navigation  CMR to evaluate LV Scar › Characteristically, patients who ALCAPA have sub-endocardial late gadolinium enhancement (LGE) of the basal anterolateral wall and sometimes papillary muscle1  CMR › Extremely valuable tool in defining coronary anatomy in adult patients who have undergone surgery as young children for congenital abnormalities › May obviate the need for invasive procedures 1. Secinaro A, Ntsinjana H, et al. Cardiovascular magnetic resonance findings in repaired anomalous left coronary artery to pulmonary artery connection (ALCAPA). Journal of Cardiovascular Magnetic Resonance. 2011. 13:27
  • 10.  Gyanendra Sharma, M.D. – Director of Echocardiogram Laboratory, Professor of Medicine, Division of Cardiology  Jayanth H. Keshavamurthy, M.D. – Assistant Professor of Radiology

Editor's Notes

  1. Aorto-pulmonary Window – Baffle to the LM starting from the aorta
  2. Aorto-pulmonary Window – Baffle to the LM starting from the aorta (mid baffle)
  3. Aorto-pulmonary Window – Baffle to the LM starting from the aorta (LM bifurcation)
  4. Sub-endocardium, anterolateral scar
  5. Sub-endocardium, anterolateral scar
  6. an extremely valuable tool in defining coronary anatomy in adult patients who have underwent surgery as young children for congenital abnormalities such as our patient who had undergone the Takeuchi procedure