Investig Magn Reson Imaging.  2015 Dec;19(4):205-211. 10.13104/imri.2015.19.4.205.

Congenital Heart Disease: a Pictorial Illustration of Putting Segmental Approach into Practice

• Yeung TH
• Park EA
• Lee YC
• Yoo JY
• Lui CY

• KMID: 2151766
• DOI: http://doi.org/10.13104/imri.2015.19.4.205

Abstract

The human heart is a complex organ in which many complicated congenital defects may happen and some of them require surgical intervention. Due to the vast complexity of varied anatomical presentations, establishing an accurate and consistent nomenclature system is utmost important to facilitate effective communication among pediatric cardiologists, cardiothoracic surgeons and radiologists. The Van Praagh segmental approach to the complex congenital heart disease (CHD) was developed in the 1960s and has been used widely as the language for describing complex anatomy of CHD over the decades. It utilizes a systematic and sequential method to describe the cardiac segments and connections which in turn allows accurate, comprehensive and unambiguous description of CHD. It can also be applied to multiple imaging modalities such as echocardiogram, cardiac CT and MRI. The Van Praagh notation demonstrates a group of three letters, with each letter representative for a key embryologic region of cardiac anatomy: the atria, ventricles and great vessels. By using a 3-steps approach, we can evaluate complex CHD precisely and have no difficulties in communicating with other medial colleague. This pictorial essay revisits the logical steps of segmental approach, followed by a pictorial illustration of its application.

Keyword

Congenital heart disease; Situs; Solitus; Ambiguous; Inversus

MeSH Terms

Congenital Abnormalities
Heart
Heart Defects, Congenital*
Humans
Logic
Magnetic Resonance Imaging

Figure

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  Fig. 1 (a) Patient with isolated ventricular septal defect, CT demonstrates example of normal eparterial right main bronchus (arrow) located behind the pulmonary artery (RPA). (b) Patient with isolated ventricular septal defect, CT demonstrates example of normal hyparterial left main bronchus (arrow) located inferior to the pulmonary artery (LPA). (c) Patient with isolated ventricular septal defect, CT demonstrates example of normal situs of great vessels with aorta (A) right posterior to the main pulmonary artery (P).

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  Fig. 2 (a) Patient with dextro-transposition, CT showing aorta (A) located right anterior to the pulmonary artery (P). (b) Patient with levotransposition, CT showing aorta (A) located left anterior to the pulmonary artery (P).

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  Fig. 3 (a) Patient with atrioventricular septal defects, coronal localizer showing visceral anatomy: bilateral hyparterial bronchi (arrows) passing inferior to bilateral pulmonary arteries (PA) and left-sided stomach. (b) Patient with atrioventricular septal defects and situs ambiguous, CT showing visceral anatomy: midline liver and multiple spleens (arrows). (c) Patient with atrioventricular septal defects, axial CT showing atrial anatomy: large atrial septal defect with common atrium. Pulmonary veins are seen draining to common atrium at midline. Cardiomegaly and dextrocardia associated with collapsed right lung are noted. (d) Patient with atrioventricular septal defects, a diastolic frame from 4-chamber cine showing that the morphological right ventricle is located on the right side of morphological left ventricle. The right ventricle contains coarse trabeculation and moderator band (arrow) along its septal wall. The left ventricle has smooth septal surface. (e) Patient with atrioventricular septal defects, a frame from short axis cine showing the coarse trabeculations in the right ventricle and the fine trabeculations of the left ventricle. A muscular ventricular septal defect is also seen (arrow). (f) Patient with atrioventricular septal defects, CT showing the normally positioned aorta (A)right posterior to the pulmonary artery which is dilated due to pulmonary hypertension. The pulmonary artery arises from the right ventricle and normal subpulmonic muscular conus (arrows) is seen. (g) Patient with atrioventricular septal defects, a frame from left ventricular outflow tract cine showing aorta arising from left ventricle. Associated normal aortomitral continuity (arrow) is noted.

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  Fig. 4 (a) Patient with congenitally corrected transposition of great vessels, coronal localizer image showing situs inversus with left-sided liver and inferior vena cava, and right-sided stomach. The right pulmonary artery (RPA) is seen crossing superior to the right main bronchus (hyparterial, arrow). Thoracic aorta (A) descends on the right side. Inferior vena cava (IVC) drains into the left-sided morphological right atrium (RA). (b) Patient with congenitally corrected transposition of great vessels, axial localizer showing pulmonary (arrow) draining into the right-sided morphological left atrium (LA). (c) Patient with congenitally corrected transposition of great vessels, coronal localizer showing superior vena cava (SVC) draining into the left-sided morphological right atrium (RA). (d) Patient with congenitally corrected transposition of great vessels, morphological right ventricle with moderator band (arrow) and coarse trabeculae is seen rightward of morphological left ventricle (D-loop). (e) Patient with congenitally corrected transposition of great vessels, aorta arising from morphological right ventricle, which contains coarse trabeculation. Subaortic muscular conus (arrow) is seen separating the arotic valve from the tricuspid valve. (f) Patient with congenitally corrected transposition of great vessels, pulmonary artery is seen arising from the morphological left ventricle, with no muscular conus. Mitropulmonic continuity is seen (arrow). (g)Patient with congenitally corrected transposition of great vessels, the aorta (A) is located right anterior of the pulmonary artery (PA), indicating D-transposition.

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  Fig. 5 (a) Baby with atrioventricular septal defects, CT with coronal reformat and lung window setting showing the branching of left and right main bronchi into upper lobe bronchus and bronchus intermedias, indicating bilateral morphological right bronchial tree and trilobed lungs. (b) Baby with atrioventricular septal defects, dextrocardia with large atrial septal defect, the atria are grossly dilated due to regurgitation. Left atrium (LA) is seen receiving the pulmonary veins (PV). Crista terminalis (arrow) is seen within the right atrium. (c) Baby with atrioventricular septal defects, morphological right ventricle (RV) is located right to the left ventricle (LV). Coarse trabeculations and moderator band along the septal wall are seen within the morphological right ventricle. Thin streak of contrast across the apical septum represents a small ventricular septal defect (arrow). (d) Baby with atrioventricular septal defects, aorta (A) with subaortic muscular conus (arrows) is seen arising from morphological right ventricle (RV). There is no fibrous continuity between tricuspid and aortic valves. (e) Baby with atrioventricular septal defects, pulmonary artery arises from left ventricle which lacks muscular conus along its outflow tract. There is fibrous continuity (arrow) between pulmonary and mitral valves. (f) Baby with atrioventricular septal defects, aorta (A) is right anterior to the pulmonary artery (PA), indicating D-transposition.

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