Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations.
Geva T,Van Praagh S,Sanders S P,Mayer J E,Van Praagh R
Journal of the American College of Cardiology
The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.
10.1016/0735-1097(91)90655-s
Aorta-right atrial tunnel.
Sai Krishna Cheemalapati,Baruah Dibya Kumar,Reddy Gangireddy Venkateswara,Panigrahi Nanda Kishore,Suman Kalagara,Kumar Palli Venkata Naresh
Texas Heart Institute journal
Aorta-right atrial tunnel is a vascular channel that originates from one of the sinuses of Valsalva and terminates in either the superior vena cava or the right atrium. The tunnel is classified as anterior or posterior, depending upon its course in relation to the ascending aorta. An origin above the sinotubular ridge differentiates the tunnel from an aneurysm of the sinus of Valsalva, and the absence of myocardial branches differentiates it from a coronary-cameral fistula. Clinical presentation ranges from an asymptomatic precordial murmur to congestive heart failure. The embryologic background and pathogenesis of this lesion are attributable either to an aneurysmal dilation of the sinus nodal artery or to a congenital weakness of the aortic media. In either circumstance, progressive enlargement of the tunnel and ultimate rupture into the low-pressure right atrium could occur under the influence of the systemic pressure.The lesion is diagnosed by use of 2-dimensional echocardiography and cardiac catheterization. Computed tomographic angiography is an additional noninvasive diagnostic tool. The possibility of complications necessitates early therapy, even in asymptomatic patients or those with a hemodynamically insignificant shunt. Available treatments are catheter-based intervention, external ligation under controlled hypotension, or surgical closure with the patient under cardiopulmonary bypass.Herein, we discuss the cases of 2 patients who had this unusual anomaly. We highlight the outcome on follow-up imaging (patient 1) and the identification and safe reimplantation of the coronary artery (patient 2).
Organogenesis of the vertebrate heart.
Miquerol Lucile,Kelly Robert G
Wiley interdisciplinary reviews. Developmental biology
Organogenesis of the vertebrate heart involves a complex sequence of events initiating with specification and differentiation of myocardial and endocardial cells in anterior lateral mesoderm shortly after gastrulation, followed by formation and rightward looping of the early heart tube. During looping, the heart tube elongates by addition of second heart field progenitor cells from adjacent pharyngeal mesoderm at the arterial and venous poles. Progressive differentiation is controlled by intercellular signaling events between pharyngeal mesoderm, foregut endoderm, and neural crest-derived mesenchyme. Regulated patterns of myocardial gene expression and proliferation within the embryonic heart drive morphogenesis of atrial and ventricular chambers, while cardiac cushions, precursors of the definitive valves, form in the atrioventricular and outflow regions. In amniotes, separate systemic and pulmonary circulatory systems arise by septation and remodeling events that divide the atria and ventricles into left and right chambers. Cardiac neural crest cells play a key role in dividing the arterial pole of the heart into the ascending aorta and pulmonary trunk. During the remodeling phase the definitive cardiac conduction system, that coordinates the heartbeat, is established. In addition, the epicardium, critical for regulated ventricular growth and development of the coronary vasculature, spreads over the surface of the heart as an epithelium from which cells invade the myocardium to give rise to diverse cell types including fibroblasts and smooth muscle cells. Cardiogenesis thus involves highly coordinated development of multiple cell types and insight into the different lineage contributions and molecular regulation of each of these steps is expanding rapidly. WIREs Dev Biol 2013, 2:17-29. doi: 10.1002/wdev.68 For further resources related to this article, please visit the WIREs website.
10.1002/wdev.68
Septal Atrioventricular Junction Region: Comprehensive Imaging in Adults.
Saremi Farhood,Hassani Cameron,Sánchez-Quintana Damián
Radiographics : a review publication of the Radiological Society of North America, Inc
The septal atrioventricular junction is a centrally located region of the heart where the septal components of the atria and ventricles meet the aortic, mitral, and tricuspid valves. Important structures in this region include the membranous septum, the central fibrous body, the Koch triangle, the inferior pyramidal space, and the base of the interventricular septum. This small area is the home of the atrioventricular node and the atrioventricular conduction axis and has enormous importance to electrophysiologists owing to its prime role in the conduction system of the heart. The atrioventricular node lies within the triangle of Koch; and the atrioventricular bundle, or bundle of His, exits the atrioventricular node and penetrates the right fibrous trigone and runs underneath the membranous septum. The septal atrioventricular junction is a common location for intracardiac shunts such as membranous and perimembranous septal defects. Imaging classification of these defects can have important implications before surgical closure, because the atrioventricular conduction axis passes along the posteroinferior margin of most perimembranous defects. Extracardiac inflammatory and malignant pathologic conditions can extend from the mediastinum toward the inferior pyramidal space in this region through the epicardial fat planes. Although the anatomic structures are complicated, the components can be shown in exquisite detail with computed tomography (CT). In this review, the anatomic boundaries and important anatomic landmarks are examined with CT and magnetic resonance imaging. Also described are the anatomic variants of the membranous septum pertinent to percutaneous aortic valve implantation, the vascular anatomic variants, and commonly encountered pathologic conditions related to the septal atrioventricular junction. RSNA, 2016.
10.1148/rg.2016160010
Hypoplastic Left Heart Syndrome: Definition, Morphology, and Classification.
World journal for pediatric & congenital heart surgery
This manuscript will provide information about hypoplastic left heart syndrome (HLHS) and related malformations, including definitions, morphology, and classification, based on the 2021 (IPCCC) and the (ICD-11). HLHS is defined as "a spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch." Functionally univentricular heart is defined as "a spectrum of congenital cardiac malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation, and another to the pulmonary circulation." The Norwood operation is synonymous with the term "Norwood (Stage 1)" and is defined as (1) creation of an aortopulmonary connection and neoaortic arch construction resulting in univentricular physiology and (2) creation of a controlled source of pulmonary blood flow with a calibrated systemic-to-pulmonary artery shunt, a right ventricle to pulmonary artery conduit, or rarely, a cavopulmonary connection. The goals of the Norwood (Stage 1) Operation are creation of (1) unobstructed systemic blood flow via aortopulmonary connection and neoaortic arch construction, (2) unobstructed coronary blood flow, (3) unobstructed flow across the atrial septum, and (4) controlled pulmonary blood flow.
10.1177/21501351221114770
Septal function during left ventricular unloading.
Moon M R,Bolger A F,DeAnda A,Komeda M,Daughters G T,Nikolic S D,Miller D C,Ingels N B
Circulation
BACKGROUND:Left ventricular (LV) unloading with mechanical support devices alters biventricular geometry and impairs right ventricular (RV) contractility, but its effect on septal systolic function remains unknown. METHODS AND RESULTS:To evaluate the effects of LV volume and pressure unloading on septal geometry and function, LV preload was abruptly reduced by clamping left atrial pressure between 0 and -2 mm Hg in seven open-chest, anesthetized dogs by use of a pressure-control servomechanism to withdraw blood from the left atrium. With left atrial pressure clamping, maximal LV pressure decreased 30 +/- 12% (mean +/- SD) (P < .0001) and LV end-diastolic cross-sectional area (determined by two-dimensional echocardiography) decreased by 53 +/- 16% (P < .0001). This caused the septum to shift toward the left (RV septal free-wall dimension increased; P < .004) and flatten (radius of curvature increased; P < .0002), while LV septal free-wall dimension fell (P < .0001). Septal end-diastolic thickness increased 23 +/- 15% (P < .0005), reflecting a decline in septal preload. Systolic septal thickening decreased (P < .002), while systolic septal output (Septal Output = Septal Thickening x Heart Rate) fell from 30 +/- 17 to 15 +/- 22 cm/min (P < .002). This was associated with movement along the septal Frank-Starling equivalent (septal output versus end-diastolic septal thickness [preload] relation) to a less productive portion of the curve. CONCLUSIONS:LV unloading not only altered interventricular septal geometry but also reduced septal systolic thickening and output, all of which may contribute to impaired RV contractility during mechanical LV support.
10.1161/01.cir.95.5.1320