Muscular ventricular septal defects: A reappraisal of the anatomy

Wenink, Arnold C. G.; Oppenheimer-Dekker, Arentje; Moulaert, A. J.

doi:10.1016/s0002-9149(79)80013-2

Cited by 45 publications

(14 citation statements)

References 13 publications

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“…Figure 2 illustrates the location of various defects. The membraneous defect is the most common type of VSD seen a surgery or autopsy, accounting for about 80% of defects [2,8,9], wherease the muscular defect comprises only 5% -20% [2,7,8,16]. Recent articles describing echocardiographic findings in septal defects report frequencies of about 60% membraneous, 32% muscular, and 3% subpulmonic types [10,12,14].…”

Section: Discussionmentioning

confidence: 99%

Diagnosis, therapy, and outcome of ventricular septal defects in the 1st year of life: A two-dimensional colour-Doppler echocardiography study

et al. 1990

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Between January 1987 and July 1989 a ventricular septal defect (VSD) as a single cardiac lesion was detected in 269 small infants aged less than 1 year. The diagnosis was achieved by two-dimensional echocardiography and Doppler colour flow mapping using subcostal, parasternal, apical, and suprasternal views. VSDs were divided into perimembraneous, muscular, malalignment, and subpulmonary defects. Septal defects in complex lesions and atrioventricular defects were excluded. In group 1 (174 infants up to 4 weeks of age, mean 10 days) 125 muscular (71.8%), 35 (20.1%) perimembraneous, 12 (6.9%) malalignment, and 2 (1.1%) subpulmonary defects were diagnosed. One baby had a combined perimembraneous and muscular defect. In another baby a malalignment defect was associated with an av-canal. In group 2 (95 infants aged 4 weeks to 1 year, mean 4.0 months), 57 (60%) muscular, 32 (33.6%) perimembraneous and 6 (6.3%) malalignment defects were found. Within the maximum observation period of 13 months, spontaneous closure occurred in 72 (42.6%) of 169 infants who had a sufficient follow up. Sixty-four had a muscular (88.9%) and 8 (11.1%) a perimembraneous defect. Surgical intervention was required in 11 patients: five perimembraneous defects were closed, one was palliated. Five infants with a malalignment defect were palliated. The malalignment defect frequently needed surgical intervention even in newborns; it never closed spontaneously. About 10% of patients with perimembraneous septal defect required surgery. Spontaneous closure rarely occurred in early infancy. Muscular VSDs were most frequent but virtually never required therapy. Spontaneous closure rate was about 50% during the 1st year of life.

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Section: Discussionmentioning

confidence: 99%

Diagnosis, therapy, and outcome of ventricular septal defects in the 1st year of life: A two-dimensional colour-Doppler echocardiography study

et al. 1990

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“…One type-A specimen with corrected transposition, subaortic stenosis and straddling tricuspid valve had the defect located posteriorly in the inlet septum, as is typical for tricuspid straddling [20] Of the remaining two type-A cases, with transposition and tricuspid atresia (both with a bulboventricular type of septum [1], one had a typical small outlet foramen leading to the outflow chamber, and the other presented with a posterior muscular defect which formed a narrow slit-shaped ostium above a prominent posteromedial muscle [36] .…”

Section: Associated Cardiac Anomaliesmentioning

confidence: 99%

The ductus arteriosus and associated cardiac anomalies in interruption of the aortic arch

1982

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SUMMARY . Seventeen heart specimens with aortic arch interruption, a ductus-dependent anomaly, were investigated . In alt specimens the cardiac anatomy favoured a preferential flow into the pulmonary artery (from which the blood could reach the descending aorta through the ductus), Ten specimens had the interruption proximal to the left subclavian artery (type B) and nine of these presented solely with an outflow tract septal defect . On the other hand, six of seven s pecimens with the interruption distal to the left subclavian artery (type A) were associated with more complex heart malformations .The ductus was studied histologically in the 12 available arches without surgical intervention . The structure of the ductal wall, the components of which could extend considerably into the descending aorta and the pulmonary artery, ranged from that seen in a normal, mature ductus with prominent intimal cushions, to a totally abnormal persistent type with no intimal cushions and with marked elastification . This variability in ductal wall structure and the distribution of ductal tissue in the descending aorta and pulmonary artery has clinical and surgical consequences . KEY WORDS : Cardiac anomalies -Ductus arteriosus -Aortic arch interruption -Anatomy and embryologyAbsence of a segment of the aortic arch is a rare anomaly which is often associated with intracardiac abnormalities, predominantly ventricular septal defects (VSDs) . The descending aorta is joined onto the ductus arteriosus and so onto the pulmonary artery .The characteristic clinical picture in a neonate with this anomaly, after a variable period of wellbelng, is of signs and symptoms related to diminished perfusion of the trunk, lower limbs and sometimes upper limbs (low blood pressure as measured in the limbs, renal insufficiency, acidosis) and increased Pulmonary blood flow (heart failure, cyanosis) . As a result of the constriction of the ductus arteriosus, the average age at death of these neonates is only 10 . There has been only a small number of reported longer survivals without surgical intervention, due to an effective collateral circulation with or without a patent ductus arteriosus [6,9, 19,33] .In this paper we present the types of aortic arch interruption and the associated cardiac anomalies of 17 post-mortem specimens . In view of the importance of the ductus arteriosus, histological examination was performed in order to determine whether from the structural characteristics spontaneous closure or persistent patency was to be expected [10] . In four cases complete serial sectioning of the arterial pole informed us about the relation of the ductus to the adjacent elastic arteries . Material, Methods, and DefinitionsThe aortic arch interruptions (17 cases of children born at term : Tables t and 2) were classified (Fig . i) as initially outlined by Celoria and Patton 141, the A segment being the aortic isthmus .

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“…The posteromedial muscle has been described to occur in many cases of transposition [19] and muscular ventricular septal defect [20]. It is considered to be the same structure as the dorsomedian muscle ridge described by Devloo-Blancquaert and Ritter [3] and as the portion of the true posterior septum and posterior median ridge in single ventricle [5].…”

Section: Discussionmentioning

confidence: 95%

“…From there, it courses towards the apex of the heart, lying in the groove between the septum and the posterior left ventricular wall [19,20].…”

Section: Anterolateral Muscle Bundle--this Bundle If Present Is Foundmentioning

confidence: 99%

“…The third feature, the so-called leftward deviation of the anterior part of the ventricular septum, has been mentioned before in relation to transposition The anterolateral and the posteromedial muscle bundles seem to be related to different types of developmental events in cardiomorphogenesis. The origin of the posteromedial muscle can be explained with reference to the relationship between the sepration of the atrioventricular canal and of the earlyembryonic ventricle: invagination of the posterior part of the atrioventricular canal produces a muscular ridge on the posterior ventricular wall, which in normal cardiac development apparently merges invisibly and in one plane with the rest of the ventricular septum, only presenting as a posteromedial muscle in case of rightward deviation of the posterior portion of this ventricular septum [19,20]. In other words, the occurrence of a posteromedial muscle can be considered indicative of some underlying incongruity in the septational processes.…”

Section: Embryologic Considerationsmentioning

confidence: 99%

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The concurrence of dimensional aortic arch anomalies and abnormal left ventricular muscle bundles

et al. 1982

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This anatomical study was designed to evaluate the concept that reduced blood flow through the embryonic preductal aorta contributes to the pathogenesis of dimensional aortic arch anomalies. For that purpose the intracardiac anatomy of 151 specimens was examined, of which 22 had an interruption, five atresia, 76 tubular hypoplasia, and 48 local coarctation of the aortic arch. Associated malformations were found in 148 specimens (98%); the remaining three (2%) had isolated local coarctation. Anomalies predisposing to reduced aortic blood flow were present in 128 specimens (85%). Among the potential obstructive factors affecting early morphogenesis, three left ventricular muscular structures seem to be particularly important: (1) the anterolateral muscle bundle, (2) the posteromedial muscle, and (3) leftward deviation of the anterior part of the ventricular septum. Obstructing combinations with these types of anomaly were identified in 77 cases (51%). The embryologic aspects of these muscular structures are discussed.

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Muscular ventricular septal defects: A reappraisal of the anatomy

Cited by 45 publications

References 13 publications

Diagnosis, therapy, and outcome of ventricular septal defects in the 1st year of life: A two-dimensional colour-Doppler echocardiography study

Diagnosis, therapy, and outcome of ventricular septal defects in the 1st year of life: A two-dimensional colour-Doppler echocardiography study

The ductus arteriosus and associated cardiac anomalies in interruption of the aortic arch

The concurrence of dimensional aortic arch anomalies and abnormal left ventricular muscle bundles

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