Pathologie coronaire de l'enfant coursDamien Bonnet Unité médico-chirurgicale de Cardiologie Congénitale et Pédiatrique Hôpital Universitaire Necker Enfants malades – APHP,

Damien Bonnet Unité médico-chirurgicale de Cardiologie Congénitale et Pédiatrique

Hôpital Universitaire Necker Enfants malades – APHP, Université Paris Descartes, Sorbonne Paris Cité IcarP Cardiology, Institut Hospitalo-Universitaire IMAGINE

Centre de Référence Maladies RaresMalformations Cardiaques Congénitales Complexes-M3C

Centre de Référence Maladies RaresMaladies Cardiaques Héréditaires- CARDIOGEN

Coronary pathology in children

Coronary arteries in animals

« Not everyone has coronary vessels » • Invertebrates : no• Amphibians : no

• Vertebrates : mammals, reptiles, avians : yes– common characteristics : pulmonary respiration and no

percutaneous respiration• Fish : coronary arteries only in:

– Larger, fast-swimming, predatory– Living in poorly oxygenated environment

Poissons

Reese DE, Circ Res 2002 ; 91 : 761-8

Development of coronary arteries : late event in cardiac morphogenesis

Semilunar valves developmentDelamination of tricuspid valve

Convergence

Endocardial cushionsTricuspid gully

Elongation of cardiac outflow tractAortic arches 4 and 6

23 40 50444230

LoopingCardiacseptation

Wedging

18

Right horn of sinus venosusPrimitive pulmonary veinAortic arches 2 and 3

Connection coro - aorta

Day

Coronary artery patterning in Tbx1-/- hearts

RCA

LCAAo

PT

Tbx1+/-

Connexin40 eGFP

Tbx1+/+ Tbx1-/-

Tbx1+/- Tbx1-/-

RCA

LCA

RCA

LCA

Ao

PT

Coronary artery patterning in Tbx1-/- hearts

PECAMTbx1+/-

Tbx1-/- PECAM

Ao

R LD

SMC

LCARCA

Coronary sinuses are formed via ingrowth of the peritruncal capillary plexus

Tomanek RJ, Angiogenesis, 2005

Hypoxia and apoptosis are correlated with the invasion of the Aorta

AoPeritruncal

Capillary plexus R LD

Penetrating plexus

LCA

• The coronary arteries are « attracted » by the aorta (subaortic domain)

• They enter the aorta to the nearest point of their epicardial course

• While avoiding the pulmonary artery (myocardial subpulmonary domain)

Embryology : the coronary arteries enter the aorta

LCAAo

PTRCA

Embryology : coronary artery patterning

• Coronary artery patterning also depends on the underlying ventricle (double discordance)

• L-loop : mirror-imaged coronary arteries

CxRCA

LAD

Conotruncal defects

• The location of the coronary ostia depends on the degree of rotation of the outflow tract (which modifies the location of the subpulmonary domain)

Ao PA

Outflow tract rotation- +

Embryonic truncus CAT DORV TOF TOF&PA Normal heart

AoAo

PAPA

PA

AoAoCAT

Detection of myocardial ischemia in children

�11

�12

�13

Abnormal epicardial course of coronary arteries

Main abnormal epicardial courses of coronary arteries

Abnormal courses considered at low risk of cardiac event

Abnormal courses considered at high risk of cardiac event

Main abnormal epicardial courses

Interarterial course of coronary arteries

25% 75%

0,1% - 0,3% of the population ( ≈ 100 000 people in France)

Two anatomical variants

Interarterial course isolated Interarterial course and intramural

30% 70%

Mechanism of myocardial ischemia

Basso C et al. JACC 2000

Sudden death mechanisms -compression of inter arterial course during effort -progressive stenosis of the inter arterial course: early atherosclerosis/remodeling/acute thrombosis -subclinical ischemia with myocardial fibrosis and arrhythmias

Serendipitously diagnosed or not

In Vivo Detection of Coronary ArteryAnomalies in Asymptomatic Athletes byEchocardiographic Screening*Paolo Zeppilli, MD; Antonio dello Russo, MD; Cesare Santini, MD;Vincenzo Palmieri, MD; Luigi Natale, MD; Alessandro Giordano, MD; andAndrea Frustaci, MD, FCCP

Background: Anomalous origin of coronary arteries (AOCA) is a rare congenital disease. Althoughit may have a benign course, it has been identified as a frequent cause of sports-related suddendeath. Unfortunately, in vivo detection ofAOCA is not easy, as individuals with this anomaly oftenare asymptomatic and show no signs of myocardial ischemia. Presently, transthoracic two-dimensional echocardiography (TTE) is the only noninvasive, widely available tool to visualize theostia and first tracts of coronary arteries.Objective: To assess the efficacy of TTE in the screening of AOCA in a large athletic population.Study design: In a prospective study, we assessed the ostia and first tracts of coronary arteries in3,650 subjects (mean age, 30±12 years) practicing different sports at various competitive levels.Subjects underwent a TTE examination in our laboratory for scientific or diagnostic purposes.Results: Technically satisfactory echocardiograms were obtained in 3,504 subjects (96%); a clearvisualization of the ostia and first tracts of both coronary arteries was obtained in 3,150 cases

(90%). Three asymptomatic athletes (0.09%) were suspected to have an AOCA; two with a rightcoronary artery origin from the left sinus, and one with a left coronary artery origin from theright sinus. Diagnosis was confirmed by coronary angiography.Conclusions: Our study indicated that AOCA is rare in asymptomatic athletes. Systematic andaccurate exploration of coronary anatomy in athletes referred for a diagnostic TTE examinationmay be useful in identifying those with AOCA. (CHEST 1998; 114:89-93)

Key words: anomalous origin of coronary artery; athletes; echocardiography; sudden death

Abbreviations: AOCA=anomalous origin of coronary arteries; LCA=left coronary artery; RCA=right coronary artery;TTE = transthoracic two-dimensional echocardiography

A nomalous origin of coronary arteries (AOCA) is a

-^**rare congenital disease found on approximately0.6 to 1% of all coronary angiograms and in 0.3% ofall autopsies.13 Although an AOCA may have a

completely benign and asymptomatic course, it hasbeen identified as a cause for sports-related andnon-sports-related sudden death in young peopleand athletes.4"6

Unfortunately, in vivo detection of AOCA is not

always easy, because subjects with this anomaly are

*From the Centro Studi di Medicina dello Sport, Istituto diMedicina Interna e Geriatria (Drs. Zeppilli, dello Russo, Santini,and Palmieri), the Istituto di Radiologia (Dr. Natale), the Istitutodi Medicina Nucleare (Dr. Giordano), and the Istituto diCardiologia (Dr. Frustaci), Universita Cattolica del Sacro Cuore,Roma, Italy.Manuscript received September 26, 1997; revision acceptedJanuary 22, 1998.Reprint requests: Dott. Andrea Frustaci, Istituto di Cardiologia,Universita Cattolica del Sacro Cuore, Largo Gemelli 8, 00168Rome, Italy

often asymptomatic, and symptoms attributable tomyocardial ischemia are observed only in about 30%of cases.4 Presently, transthoracic two-dimensionalechocardiography (TTE) offers a reliable, noninva¬sive means of visualizing the ostia and first tracts ofboth coronary arteries, especially in children andyoung adults.7 Visualization of these structures byTTE is easier in athletes because of their favorablechest conformation, the prolonged diastolic time dueto bradycardia, and a real increase in coronary arterysize due to training.8"10 The real prevalence ofAOCAin athletes is not known but it is probably very low;Pelliccia et al9 found no cases of AOCA in a largegroup of asymptomatic elite athletes prospectivelystudied by TTE.

Since 1986, in our Sports Medicine Laboratory,routine TTE examination of all subjects practicingsports has included a systematic search of the ostiaand the first tracts of both coronary arteries. Usingthis approach, we were able to identify three athletes

CHEST/114/1/JULY, 18

1998 by the American College of Chest Physicians by guest on September 11, 2011chestjournal.chestpubs.orgDownloaded from

In Vivo Detection of Coronary ArteryAnomalies in Asymptomatic Athletes byEchocardiographic Screening*Paolo Zeppilli, MD; Antonio dello Russo, MD; Cesare Santini, MD;Vincenzo Palmieri, MD; Luigi Natale, MD; Alessandro Giordano, MD; andAndrea Frustaci, MD, FCCP

Background: Anomalous origin of coronary arteries (AOCA) is a rare congenital disease. Althoughit may have a benign course, it has been identified as a frequent cause of sports-related suddendeath. Unfortunately, in vivo detection ofAOCA is not easy, as individuals with this anomaly oftenare asymptomatic and show no signs of myocardial ischemia. Presently, transthoracic two-dimensional echocardiography (TTE) is the only noninvasive, widely available tool to visualize theostia and first tracts of coronary arteries.Objective: To assess the efficacy of TTE in the screening of AOCA in a large athletic population.Study design: In a prospective study, we assessed the ostia and first tracts of coronary arteries in3,650 subjects (mean age, 30±12 years) practicing different sports at various competitive levels.Subjects underwent a TTE examination in our laboratory for scientific or diagnostic purposes.Results: Technically satisfactory echocardiograms were obtained in 3,504 subjects (96%); a clearvisualization of the ostia and first tracts of both coronary arteries was obtained in 3,150 cases

(90%). Three asymptomatic athletes (0.09%) were suspected to have an AOCA; two with a rightcoronary artery origin from the left sinus, and one with a left coronary artery origin from theright sinus. Diagnosis was confirmed by coronary angiography.Conclusions: Our study indicated that AOCA is rare in asymptomatic athletes. Systematic andaccurate exploration of coronary anatomy in athletes referred for a diagnostic TTE examinationmay be useful in identifying those with AOCA. (CHEST 1998; 114:89-93)

Key words: anomalous origin of coronary artery; athletes; echocardiography; sudden death

Abbreviations: AOCA=anomalous origin of coronary arteries; LCA=left coronary artery; RCA=right coronary artery;TTE = transthoracic two-dimensional echocardiography

A nomalous origin of coronary arteries (AOCA) is a

-^**rare congenital disease found on approximately0.6 to 1% of all coronary angiograms and in 0.3% ofall autopsies.13 Although an AOCA may have a

completely benign and asymptomatic course, it hasbeen identified as a cause for sports-related andnon-sports-related sudden death in young peopleand athletes.4"6

Unfortunately, in vivo detection of AOCA is not

always easy, because subjects with this anomaly are

*From the Centro Studi di Medicina dello Sport, Istituto diMedicina Interna e Geriatria (Drs. Zeppilli, dello Russo, Santini,and Palmieri), the Istituto di Radiologia (Dr. Natale), the Istitutodi Medicina Nucleare (Dr. Giordano), and the Istituto diCardiologia (Dr. Frustaci), Universita Cattolica del Sacro Cuore,Roma, Italy.Manuscript received September 26, 1997; revision acceptedJanuary 22, 1998.Reprint requests: Dott. Andrea Frustaci, Istituto di Cardiologia,Universita Cattolica del Sacro Cuore, Largo Gemelli 8, 00168Rome, Italy

often asymptomatic, and symptoms attributable tomyocardial ischemia are observed only in about 30%of cases.4 Presently, transthoracic two-dimensionalechocardiography (TTE) offers a reliable, noninva¬sive means of visualizing the ostia and first tracts ofboth coronary arteries, especially in children andyoung adults.7 Visualization of these structures byTTE is easier in athletes because of their favorablechest conformation, the prolonged diastolic time dueto bradycardia, and a real increase in coronary arterysize due to training.8"10 The real prevalence ofAOCAin athletes is not known but it is probably very low;Pelliccia et al9 found no cases of AOCA in a largegroup of asymptomatic elite athletes prospectivelystudied by TTE.

Since 1986, in our Sports Medicine Laboratory,routine TTE examination of all subjects practicingsports has included a systematic search of the ostiaand the first tracts of both coronary arteries. Usingthis approach, we were able to identify three athletes

CHEST/114/1/JULY, 18


3/31500.09%

Pediatric Cardiology

Major Coronary ArteryAnomalies in a Pediatric Population:Incidence and Clinical ImportanceJulie A. Davis, MD, Frank Cecchin, MD, FACC, Thomas K. Jones, MD, FACC,Michael A. Portman, MD, FACCSeattle, Washington

OBJECTIVES We sought to prospectively determine the incidence and clinical significance of majorcoronary artery anomalies in asymptomatic children using transthoracic two-dimensionalechocardiography.

BACKGROUND Anomalous origins of the left main coronary artery (ALMCA) from the right sinus ofValsalva or anomalous origins the right coronary artery (ARCA) from the left sinus are rarelydiagnosed in children and can cause sudden death, especially in young athletes. Because mostpatients are asymptomatic, the diagnosis is often made post mortem. No study to date hasprospectively identified anomalous coronary arteries in asymptomatic children in the generalpopulation.

METHODS After serendipitously identifying an index case with ALMCA, we examined proximalcoronary artery anatomy in children with otherwise anatomically normal hearts who werereferred for echocardiography. In those diagnosed with ALMCA or ARCA, we performedfurther tests.

RESULTS Within a three-year period, echocardiograms were obtained in 2,388 children and adoles-cents. Four children (0.17%) were identified with anomalous origin of their coronary arteries,and angiograms, exercise perfusion studies and/or stress tests were then performed. OneARCA patient had decreased perfusion in the right coronary artery (RCA) perfusion area andshowed ventricular ectopy on electrocardiogram (ECG) at rest that diminished but did notresolve with exercise. A second patient with ALMCA had atrial tachycardia immediately afterexercise, with inferior and lateral ischemic changes on ECG and frequent junctional and/orventricular premature complexes both at rest and recovery.

CONCLUSIONS This study demonstrates that although anomalous origins of coronary arteries are rare inasymptomatic children, the prevalence is greater than that found in other prospective studies.Ischemia can occur with both ALMCA and ARCA even though patients remain asymp-tomatic. Because of the high risk of sudden cardiac death, aggressive surgical managementand close follow-up are necessary. (J Am Coll Cardiol 2001;37:593–7) © 2001 by theAmerican College of Cardiology

Anomalous origins of the left main coronary artery(ALMCA) from the right sinus of Valsalva or anomalousorigins the right coronary artery (ARCA) from the left sinusof Valsalva are rare congenital anomalies with a combinedincidence ranging from 0.17% in autopsy studies to 1.2% inpatients angiographically evaluated (1,2). Sudden deathoccurs with both anomalies, most notably in young athleticindividuals (3–5). The diagnosis is often first made postmortem because most of these patients are asymptomatic

See page 598

(6), with cardiovascular symptoms (e.g., chest pain, exer-tional dyspnea, syncope or dizziness) occurring in only 18%to 30% of patients (7,8). These anomalies can be detected by

transthoracic two-dimensional echocardiography (TTE),which offers a safe, noninvasive way of examining theproximal coronary artery anatomy (9).

Indeed, few studies to date have prospectively identifiedALMCA or ARCA in children and adolescents (9–12).Those that have done so identified patients either becausethey were symptomatic (9,10) or because they were athletesrequired to undergo preparticipation echocardiographicscreening (11,12). No studies have identified anomalouscoronary arteries in asymptomatic children and adolescentsin the general population. Therefore, our study was de-signed to prospectively determine the incidence and clinicalsignificance of major coronary artery anomalies in otherwiseasymptomatic children and adolescents by using TTE.

METHODS

Patients. An index case with ALMCA was serendipitouslyidentified and reported at our institution using TTE (13).Subsequently, between February 1997 and December 1999,

From the Department of Cardiology, Children’s Hospital and Regional MedicalCenter, Seattle, Washington.

Manuscript received May 11, 2000; revised manuscript received August 1, 2000,accepted October 12, 2000.

Journal of the American College of Cardiology Vol. 37, No. 2, 2001© 2001 by the American College of Cardiology ISSN 0735-1097/01/$20.00Published by Elsevier Science Inc. PII S0735-1097(00)01136-0

4/23880.17%

Left coronary artery from the right ostium with inter arterial course


Left coronary artery from the right ostium with inter arterial course and intramural origin



RCA from left ostium





Anatomical repair

Anatomical repair

Anatomical repair

Anatomical repair: result

Septal course of LAD in the conal septum



Intraseptal left anterior descending coronary artery

Septal course of LADin the conal septum

Intraseptal left anterior descending coronary artery

Brothers JA et al. AJR 2015

CT after arterial switch operation for TGA Abnormal course

Acquired inter arterial course of coronary arteries

CT after arterial switch operation for TGA Intramural left main CA


CT after arterial switch operation for TGA Retroaortic course LMCA

Compression of CA by a stent



DOI 10.1378/chest.112.3.842 1997;112;842-843Chest

GandjbakhchLacombe, Michel Rigaud, Jean-Pierre Bourdarias and Iradj Jean-Frédéric Patrat, Guillaume Jondeau, Olivier Dubourg, Pascal During Primary Pulmonary HypertensionLeft Main Coronary Artery Compression

http://chestjournal.chestpubs.org/content/112/3/842

services can be found online on the World Wide Web at: The online version of this article, along with updated information and

ISSN:0012-3692)http://chestjournal.chestpubs.org/site/misc/reprints.xhtml(

prior written permission of the copyright holder.this article or PDF may be reproduced or distributed without theDundee Road, Northbrook, IL 60062. All rights reserved. No part of Copyright1997by the American College of Chest Physicians, 3300Physicians. It has been published monthly since 1935.

is the official journal of the American College of ChestChest


Fig. 1 A 28 year old male athlete with a malignant course ofthe RCA between the aorta and the pulmonary trunk with slightnarrowing of the RCA origin on 3D-MRCA (a) and MDCTA(b). Bridging (intramuscular course) of the mid-LAD on 3D-

MRCA (c) and MDCTA (d). The super Bruce tread-mill testand scintigraphy were negative. No X-ray coronary angiogra-phy was performed

Fig. 2 A 21 year old male non-athlete with a high ventralaorta origin RCA on 3D-MRCA (a: axial image, b: coronaloblique image) and confirmed on MDCTA (c). The standard

Bruce tread-mill test was negative. No scintigraphy or X-raycoronary angiography was performed

706 Int J Cardiovasc Imaging (2010) 26:701–710

123

Fig. 3 A 46 year old male athlete with left sinus origin andmalignant course of the RCA between the aorta and pulmonarytrunk on 3D-MRCA (a) and MDCTA (b). An additionalsignificant soft-plaque stenosis of the mid-RCA (70% lumennarrowing) on MDCTA (c) was missed on 3D-MRCA due tolimited coverage. The Agatston coronary calcium score was

negative. The super Bruce treadmill test was positive. Noscintigraphy was performed. An X-ray coronary angiogramshowed an 80% lumen stenosis of the mid-RCA (d).Percutaneous coronary intervention of mid-RCA with baremetal stent implantation was performed (e)

Fig. 4 A 35 year old male athlete with a ventral rotation ofRCA originating from the right coronary sinus withoutmalignant course but with possible stenosis on 3D-MRCA(a). The ventral rotation origin of the RCA was confirmed, but

no stenosis was seen on MDCTA (b). The super Brucetreadmill test was negative. No scintigraphy or X-ray coronaryangiography was performed

Int J Cardiovasc Imaging (2010) 26:701–710 707

123

Sivakumar,K, et al. Tex Heart Inst J. 2010; 37(1): 95–98.


Abnormal originof coronary arteries

Abnormal origin from the aorta High take off left coronary artery

Abnormal origin from the aorta High take off left coronary artery

Abnormal origin from the aorta High take off of right coronary artery

Abnormal origin from the pulmonary artery : ALCAPA






�64


Abnormal origin from the pulmonary artery : ARCAPA





Coronary artery from the left ventricle

Abnormal origin from the pulmonary artery : ALCAPA surgery

Abnormal origin from the pulmonary artery : ALCAPA Mitral regurgitation

Coronary fistulae

Coronary fistulae

Coronary fistulae

Coronary fistulae

Coronary fistulae

Coronary fistulae

Pre-embolization Post-embolization

Coronary fistulae

Coronary fistulae in PA-IVS & HLHS

Rares anomalies

GACI

SVAS

Hypercholesterolemia

Acquired coronary anomaliesKawasaki disease

Post-operative coronary obstructions

Kawasaki disease : Key points 1

1. Kawasaki disease (KD) is an acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age.

2. KD is now the most common cause of acquired heart disease in children in developed countries.

3. In the absence of pathognomonic tests, the diagnosis continues to rest on the identification of principal clinical findings and the exclusion of other clinically similar entities with known causes.

Kawasaki disease : Key points 21. Timely initiation of treatment with intravenous immunoglobulin

(IVIG) has reduced the incidence of coronary artery aneurysms defined from absolute luminal dimensions from 25% to ≈4%. Ongoing studies with additional therapies have not substantially reduced this residual risk.

2. The long-term prognosis is determined by the initial and current level of coronary artery involvement. Certain subsets of patients are at risk for myocardial ischemia from coronary artery thrombosis and stenoses.

3. Medical management of such patients hinges on judicious use of thromboprophylaxis and vigilance to identify evolving stenoses. Invasive revascularization procedures might be required for selected patients.

Brian W. McCrindle et al. Circulation. 2017;135:e927-e999

Natural history of coronary artery abnormalities

Epicardial coronary artery (right) and epicardial vein (left) from a 19-month-old child who died 10 months after Kawasaki disease onset.


Luminal myofibroblastic proliferation

Thrombosis of giant coronary artery aneurysms in Kawasaki disease

Clinical criteria for the diagnosis of Kawasaki disease

Classic KD is diagnosed in the presence of fever for at least 5 days (the day of fever onset is taken to be the first day of fever) together with at least 4 of the 5 following principal clinical features:

1. Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa 2. Bilateral bulbar conjunctival injection without exudate 3. Rash: maculopapular, diffuse erythroderma, or erythema multiforme-like 4. Erythema and edema of the hands and feet in acute phase and/or periungual desquamation in subacute phase 5. Cervical lymphadenopathy (≥1.5 cm diameter), usually unilateral

Clinical features of classic Kawasaki disease.





�98


Evaluation of suspected incomplete Kawasaki disease

Kawasaki disease, coronary dilatation & aneurysms

�103

1. No involvement: Always <22. Dilation only: 2 to <2.5; or if initially <2, a decrease in Z score during follow-up ≥ 13. Small aneurysm: ≥ 2.5 to <54. Medium aneurysm: ≥ 5 to <10, and absolute dimension <8 mm5. Large or giant aneurysm: ≥ 10, or absolute dimension ≥ 8 mm

Z-Score Classification in Kawasaki disease

Mean and prediction limits for 2 and 3 SDs for size of (A) LAD,

(B) proximal RCA, and (C) LMCA according to body

Jane W. Newburger et al. Circulation. 2004;110:2747-2771

Recommendations for Cardiovascular Assessment for Diagnosis and Monitoring During the Acute Illness

1. Echocardiography should be performed when the diagnosis of KD is considered, but unavailability or technical limitations should not delay treatment.

2. Coronary arteries should be imaged, and quantitative assessment of luminal dimensions, normalized as Z scores adjusted for body surface, should be performed.

3. For uncomplicated patients, echocardiography should be repeated both within 1 to 2 weeks and 4 to 6 weeks after treatment.

4. For patients with important and evolving coronary artery abnormalities (Z score > 2.5) detected during the acute illness, more frequent echocardiography (at least twice per week) should be performed until luminal dimensions have stopped progressing to determine the risk for and presence of thrombosis.

5. To detect coronary artery thrombosis, it may be reasonable to perform echocardiography for patients with expanding large or giant aneurysms twice per week while dimensions are expanding rapidly and at least once weekly in the first 45 days of illness, and then monthly until the third month after illness onset, because the failure to escalate thromboprophylaxis in time with the rapid expansion of aneurysms is a primary cause of morbidity and mortality.

Recommendations for Initial Treatment With IVIG and ASA

1. Patients with complete KD criteria and those who meet the algorithm criteria for incomplete KD should be treated with high-dose IVIG (2 g/kg given as a single intravenous infusion) within 10 days of illness onset but as soon as possible after diagnosis.

2. It is reasonable to administer IVIG to children presenting after the 10th day of illness (ie, in whom the diagnosis was missed earlier) if they have either persistent fever without other explanation or coronary artery abnormalities together with ongoing systemic inflammation, as manifested by elevation of ESR or CRP (CRP > 3.0 mg/dL).

3. Administration of moderate- (30–50 mg/kg/d) to high-dose (80–100 mg/kg/d ) ASA is reasonable until the patient is afebrile, although there is no evidence that it reduces coronary artery aneurysms.

4. IVIG generally should not be administered to patients beyond the tenth day of illness in the absence of fever, significant elevation of inflammatory markers, or coronary artery abnormalities.

5. The ESR is accelerated by IVIG therapy and therefore should not be used to assess response to IVIG therapy. A persistently high ESR alone should not be interpreted as a sign of IVIG resistance.

Recommendations for Adjunctive Therapies for Primary Treatment

1. Single-dose pulse methylprednisolone should not be administered with IVIG as routine primary therapy for patients with KD.

2. Administration of a longer course of corticosteroids (eg, tapering over 2–3 weeks), together with IVIG 2 g/kg and ASA, may be considered for treatment of high-risk patients with acute KD, when such high risk can be identified in patients before initiation of treatment

Recommendations for Adjunctive Therapies for Primary Treatment

1. It is reasonable to administer a second dose of IVIG (2 g/kg) to patients with persistent or recrudescent fever at least 36 hours after the end of the first IVIG infusion .

2. Administration of high-dose pulse steroids usually methylprednisolone 20–30 mg/kg intravenously for 3 days, with or without a subsequent course and taper of oral prednisone) may be considered as an alternative to a second infusion of IVIG or for retreatment of patients with KD who have had recurrent or recrudescent fever after additional IVIG .

3. Administration of a longer (eg, 2–3 weeks) tapering course of prednisolone or prednisone, together with IVIG 2 g/kg and ASA, may be considered in the retreatment of patients with KD who have had recurrent or recrudescent fever after initial IVIG treatment.

4. Administration of infliximab (5 mg/kg) may be considered as an alternative to a second infusion of IVIG or corticosteroids for IVIG-resistant patients.

5. Administration of cyclosporine may be considered in patients with refractory KD in whom a second IVIG infusion, infliximab, or a course of steroids has failed.

6. Administration of immunomodulatory monoclonal antibody therapy (except TNF-α blockers), cytotoxic agents, or (rarely) plasma exchange may be considered in highly refractory patients who have failed to respond to a second infusion of IVIG, an extended course of steroids, or infliximab.

Recommendations for Prevention of Thrombosis During the Acute Illness

1. Low-dose ASA (3–5 mg/kg/d) should be administered to patients without evidence of coronary artery changes until 4 to 6 weeks after onset of illness.

2. For patients with rapidly expanding coronary artery aneurysms or a maximum Z score of ≥ 10, systemic anticoagulation with LMWH or warfarin (international normalized ratio target 2.0–3.0) in addition to low-dose ASA is reasonable.

3. For patients at increased risk of thrombosis, for example, with large or giant aneurysms (≥ 8 mm or Z score ≥ 10) and a recent history of coronary artery thrombosis, “triple therapy” with ASA, a second antiplatelet agent, and anticoagulation with warfarin or LMWH may be considered.

4. Ibuprofen and other nonsteroidal antiinflammatory drugs with known or potential involvement of cyclooxygenase pathway may be harmful in patients taking ASA for its antiplatelet effects.

Acquired coronary anomaliesKawasaki disease

Post-operative coronary obstructions

AimsofcoronaryarteryevaluationinTGA

• Beforearterialswitchoperation– Nointerest(exceptforthechoiceofthesurgeon)

• CAstatusafterarterialswitchoperation– EarlypostoperativecontrolafterCAtransfer

• Incasesofdifficulttransfer:intramuralcourse• Post-operativemyocardialischemia

– Midtermcontrol• PrevalenceofCAobstruction=5to7%(Legendreetal.Circulation2003;108suppl1:II186-90)

• Canbefoundinasymptomaticchildren

�113

Coronary artery distribution and mortality after ASO Pasquali, 2002

Type B Intramural course

• Single ostium and intramural course increase the risk of postoperative death

• Postoperative mortality is increased in patients with abnormal coronary artery distribution (OR 1,7).

�114Type C intramural

LatecoronaryarteryanomaliesafterASO

• Minordistortionduringtransfer?:endothelium,intimalproliferation,…

• Evolution:compression,stretching

• Outcome– Suddendeathandmyocardialinfarction

• Before6monthsofage– Asymptomaticmyocardialischemia

• Balancebetweenintimalproliferationanddevelopmentofcollateralcirculation

– Coronaryocclusionwithoutischemia

IndicationstocontrolCAafterASO

• Anytimeincaseofpatentmyocardialischemia

• Systematicscreeninginschoolagechildren

• Whathastobedetected?– CAobstruction:imaging

• Prevalencevsriskofdiagnosticprocedure

– Myocardialischemia:function• Negativepredictivevalue?(>50%falsenegative)

• PositivepredictivevalueOK(fewfalsepositive)

Late coronary artery anomalies after ASO

64-slice CT after arterial switch operation for TGA Intramural left main CA

64-slice CT after arterial switch operation for TGA LMCA stenosis

64-slice CT after arterial switch operation for TGA Abnormal course

64-slice CT after arterial switch operation for TGA LMCA retro-aortic course

64-slice CT after arterial switch operation for TGA RCA compression

How to treat CA obstructions ?

CA surgical angioplasty Raisky et al. Eur J Cardiothorac Surg 2007;31:895-9 PTCA Kampmann et al. Ann Thorac Surg 2005;80:1641-6

Conclusion

• WidevarietyofCAanomalies• Recentadvancesinnoninvasiveimagingnchildren• Newinsightsinthemechanismsofpost-ASOcoronaryobstruction• StillasurgicalchallengeinCHD

Documents

Pathologie coronaire de l'enfant coursDamien Bonnet Unité médico-chirurgicale de Cardiologie Congénitale et Pédiatrique Hôpital Universitaire Necker Enfants malades – APHP,