TY - JOUR
T1 - Two-dimensional echocardiographic anatomy of atrioventricular alignment discordance with situs concordance
AU - Geva, Tal
AU - Sanders, Stephen P.
AU - Ayres, Nancy A.
AU - O'Laughlin, Martin P.
AU - Parness, Ira A.
N1 - Funding Information:
METHODS AND RESULTS Patient 1. A 15-month-old girl was noted to have cyanosis and a holosystolic murmur at birth. The ECG showed only left-axis deviation. The chest radiograph revealed visceral situs solitus, levocardia, a mildly enlarged cardiac silhouette, and increased pulmonary vascular markings. The echocardiograms howeds itussolitus of the visceraa nd atria. Right juxtaposition of the atria1 appendagesw ass een from the subxiphoid four-chamber and parasternal short-axis views. The right-sided right atrium received the superior and inferior venae cavae and the coronary sinus. The right atrium opened into a right-sided left ventricle via a right-sided, inferior, and slightly posterior mitral valve (Fig. 1, A to C). The mitral valve had two leaflets with normal chordal attachments into two well-spacedl eft ventricular papillary muscles.T he pulmonary veins drained normally into a hypoplastic left-sided left atrium that opened into a left-sided right ventricle via a markedly hypoplastic, left-sided, anterior, and superior tricuspid valve (Fig. 1, A). Pulsed and color-flow Doppler mapping showedf low from the left atrium into the right ventricle through the tricuspid valve. The diminutive tricuspid valve had chordal attachments to the interventricular septum without straddling. The right ventricle was superior and to the left relative to the left ventricle with a horizontal interventricular septum resulting in superoinferior ventricles (Fig. 1, A). The right ventricular sinus (inflow) was hypoplastic (Fig. 1, A). The infundibulum, which gave rise to the pulmonary artery, wasw ell developedand positioned to the left of the right ventricular inflow (sinus) (Fig. 1, C). Hence, a D-loop right ventricle was present. The AV alignments were discordant (right atrium to left ventricle and left atrium to right ventricle) and the AV situs were concordant (situs solitus of the atria and D-loop ventricles). The aorta originated from the left ventricle with aortic-to-mitral contiguity via an elongated intervalvular fibrosa, as seen from the subxiphoid (Fig. 1, B) and parasternal views. The pulmonary valve wass lightly anterior and well to the left of the aortic valve and was supported by the well-developed subpulmonary infundibulum (Fig. 1, C). Hence ventriculoar-
PY - 1993/2
Y1 - 1993/2
N2 - Relationships between the atria and the ventricles are commonly described in tems of either atrioventricular (AV) alignments (or connections) or AV situs (i.e., the type of atrial situs and the type of ventricular situs or ventricular loop). With either method of analysis, only one type of AV relationship (situs or alignment) is diagnosed specifically and is considered to be predictive of the other type of AV relationship. The two-dimensional echocardiographic characteristics of two patients with incongruent AV situs and alignments are described for the first time. Patient 1 had situs solitus of the viscera and atria (S), ventricular D-loop (D), and solitus normally related great arteries (S), or {S,D,S} segmental combination with concordant AV situs. However, the right-sided right atrium drained into the inferior and right-sided left ventricle, and the left-sided left atrium drained into the superior and left-sided right ventricle. Hence AV alignment discordance was present. Patient 2 had visceroatrial situs solitus (S), ventricular D-loop (D), and double-outlet right ventricle with a rightward aortic valve (D), or {S,D,D} segmental set (AV situs concordance). Similar to patient 1, AV alignment discordance was demonstrated. In both patients the diagnosis was established by two-dimensional echocardiography and subsequently confirmed by cardiac catheterization. The key to accurate echocardiographic diagnosis of this congenital heart disease was independent analysis of the AV alignments and the segmental situs of the three main cardiac segments. This diagnosis was determined by scanning from the parasternal, subxiphoid, and apical windows. The cases reported here illustrate that for complete and accurate anatomic descriptions of congenitally malformed hearts, both alignments and situs must be independently analyzed and reported as separate variables. Such analysis can be achieved readily by two-dimensional echocardiography.
AB - Relationships between the atria and the ventricles are commonly described in tems of either atrioventricular (AV) alignments (or connections) or AV situs (i.e., the type of atrial situs and the type of ventricular situs or ventricular loop). With either method of analysis, only one type of AV relationship (situs or alignment) is diagnosed specifically and is considered to be predictive of the other type of AV relationship. The two-dimensional echocardiographic characteristics of two patients with incongruent AV situs and alignments are described for the first time. Patient 1 had situs solitus of the viscera and atria (S), ventricular D-loop (D), and solitus normally related great arteries (S), or {S,D,S} segmental combination with concordant AV situs. However, the right-sided right atrium drained into the inferior and right-sided left ventricle, and the left-sided left atrium drained into the superior and left-sided right ventricle. Hence AV alignment discordance was present. Patient 2 had visceroatrial situs solitus (S), ventricular D-loop (D), and double-outlet right ventricle with a rightward aortic valve (D), or {S,D,D} segmental set (AV situs concordance). Similar to patient 1, AV alignment discordance was demonstrated. In both patients the diagnosis was established by two-dimensional echocardiography and subsequently confirmed by cardiac catheterization. The key to accurate echocardiographic diagnosis of this congenital heart disease was independent analysis of the AV alignments and the segmental situs of the three main cardiac segments. This diagnosis was determined by scanning from the parasternal, subxiphoid, and apical windows. The cases reported here illustrate that for complete and accurate anatomic descriptions of congenitally malformed hearts, both alignments and situs must be independently analyzed and reported as separate variables. Such analysis can be achieved readily by two-dimensional echocardiography.
UR - http://www.scopus.com/inward/record.url?scp=0027509285&partnerID=8YFLogxK
U2 - 10.1016/0002-8703(93)90026-6
DO - 10.1016/0002-8703(93)90026-6
M3 - Article
C2 - 8427141
AN - SCOPUS:0027509285
VL - 125
SP - 459
EP - 464
JO - American Heart Journal
JF - American Heart Journal
SN - 0002-8703
IS - 2 PART 1
ER -