Ventricular inversion without transposition of the great vessels in situs inversus

Espino-Vela, Jorge; Cruz, María V. de la; Muñóz-Castellanos, Luis; Plaza, L; Attié, Fause

doi:10.1136/hrt.32.3.292

Cited by 42 publications

(14 citation statements)

References 7 publications

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“…The hemodynamic data in this patient and the surgical outcome of the case of Dunkman et al . [ A review of the medical literature also revealed three cases of isolated ventricular inversion in situs inversus (Table 2) [2,3] . A detailed description of the case of Fox et al .…”

Section: Discussionmentioning

confidence: 97%

“…[3] is not available ; however, both of the cases of Espino-Vela et al . [2] had complex associated defects such as total anomalous pulmonary venous connection, mitral stenosis or atresia, and valvar and infundibular pulmonic stenosis . Both of these cases presented with hypoxic or cyanotic spells, dyspnea, and decreased-to-normal pulmonary vascular markings on chest x-ray .…”

Section: Discussionmentioning

confidence: 98%

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Isolated ventricular inversion: Two-dimensional echocardiographic findings and a review of the literature

et al. 1984

Pediatr Cardiol

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In a cyanotic newborn infant, the diagnosis of isolated ventricular inversion in situs solitus was made from the two-dimensional echocardiographic examination and later confirmed at cardiac catheterization and surgery. The four-chamber views were especially useful for identifying the normal atrial situs and discordant atrioventricular connections in this infant. The normal relationships of the great arteries to each other (aortic valve rightward, posterior, and inferior to the pulmonary valve; great arteries coiled around each other) and to the ventricles (mitral-aortic fibrous continuity, well-developed subpulmonic conus) were well defined in the subcostal views. A review of the medical literature revealed a total of 12 cases of isolated ventricular inversion, most of which were diagnosed only at postmortem examination. The two-dimensional echocardiogram provides a rapid, safe technique for prompt diagnosis in these very sick cyanotic infants.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 98%

Isolated ventricular inversion: Two-dimensional echocardiographic findings and a review of the literature

et al. 1984

Pediatr Cardiol

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“…More than 40 cases of isolated or complex forms of discordant atrioventricular with concordant ventriculo-arterial connections have been reported, including cases with heterotaxy. [1][2][3][4][5][6][7][8]13 The challenge of diagnosing this rare cardiac defect, especially if it is part of a complex CHD, has not changed since its first description. The main problem in all historical patients was that the correct anatomical diagnosis was only made at autopsy.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Reports about this complex cardiac defect remain rare and are mainly case descriptions and small patient series, often lacking information on long-term follow-up. [3][4][5][6][7][8] Atrial switch operation by Mustard or Senning technique is the surgical method of choice. [3][4][5][6][7][8] We report here a series of eight patients with discordant atrioventricular and concordant ventriculoarterial connections by discussing the difficulties in correctly describing the anatomy and obtain an accurate pre-operative diagnosis in these complex settings.…”

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confidence: 99%

Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome

et al. 2015

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Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors. Discussion Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.

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“…Three cases previously designated as having "isolated ventricular inversion"" or "ventricular inversion without transposition'2 13 have not been included since they definitely or almost certainly had an absent atrioventricular connexion.…”

Section: Discussionmentioning

confidence: 99%

Mustard's operation for patients with ventriculoarterial concordance. Report of two cases and a cautionary tale.

Arciprete¹,

Macartney²,

Leval³

et al. 1985

Heart

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suMMARY Three patients with ventriculoarterial concordance and an abnormal atrioventricular connexion were investigated. One patient had total anomalous pulmonary venous connexion to a left vertical vein. In 1972 this was repaired and a ventricular septal defect was closed, but the presence of atrioventricular discordance was missed and the patient died. In the other two patients, Mustard's operation resulted in survival. One patient had situs solitus and atrioventricular discordance with an associated ventricular septal defect, which was closed. The other had left isomerism (quasi solitus) with an ambiguous atrioventricular connexion (quasi discordant). A previously noted ventricular septal defect had spontaneously closed.Intra-atrial baffle procedures (venous switches) such as Mustard or Senning operations are normally used to compensate congenitally determined single switches in the circulation. By far the commonest of these is complete transposition, in which the patient has a single switch (ventriculoarterial discordance with atrioventricular concordance). A much rarer indication for venous switching occurs when the single switch is at atrioventricular level and there is no switch at ventriculoarterial level-that is, there is ventriculoarterial concordance. In fact, only eight such operations have been hitherto described. [1][2][3][4][5][6] The purpose of this-report is to document two further examples and to illustrate the danger of an incorrect preoperative diagnosis in a third. 14 November 1984 ial concordance, a ventricular septal defect, and total anomalous pulmonary venous connexion to a left vertical vein. The ventricles were normally related for atrioventricular discordance, but the great arteries were side by side with the aorta to the right.On physical examination, she had moderate cyanosis and finger clubbing. There was clinical hypertrophy of both ventricles and a systolic thrill at the lower left sternal border. On auscultation, the first heart sound was normal, the second heart sound was split, and there was a pansystolic murmur at the lower left sternal border. There was no diastolic murmur and no sign of heart failure. Her chest x ray film showed a large heart with increased pulmonary vascular markings and a wide mediastinum, and her electrocardiogram showed sinus rhythm and right atrial hypertrophy with a PR interval of 0-16 s, a QRS interval of 0-0 s, and a somewhat atypical right bundle branch block pattern.

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Ventricular inversion without transposition of the great vessels in situs inversus

Cited by 42 publications

References 7 publications

Isolated ventricular inversion: Two-dimensional echocardiographic findings and a review of the literature

Isolated ventricular inversion: Two-dimensional echocardiographic findings and a review of the literature

Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome

Mustard's operation for patients with ventriculoarterial concordance. Report of two cases and a cautionary tale.

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