Concordant Ventriculoarterial Connections Research Articles

Transposition physiology in the setting of concordant ventriculo-arterial connections.

To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re-interventions, for the rare instances of transposition physiology with concordant ventriculo-arterial connections. We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo-arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Among reported cases, just over two-thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four-fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one-fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multistaged. Overall mortality was 25%, with one-fifth of patients requiring pacemakers for surgically-induced heart block. The majority of survivors were in good functional state. The flow patterns produced by discordant atrioventricular and concordant ventriculo-arterial connections remain an important, albeit rare, indication for atrial redirection or hemi-Mustard's procedure with bidirectional Glenn. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long-term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits.

Third Atrial Chamber

A 10-MONTH-OLD BOY (weight: 5.8 kg; height: 66 cm; body mass index: 13) was taken to the cardiac catheterization laboratory before the creation of a bidirectional Glenn shunt. At 4 days of age, transthoracic echocardiography done for the evaluation of persistent cyanosis revealed heterotaxy (left atrial isomerism), situs ambiguous with levocardia, bilateral superior vena cava, atrial septal defect (9 mm), partial anomalous right pulmonary venous return to the morphologic right atrium, discordant atrioventricular connection and concordant ventriculoarterial connection, with a hypoplastic right ventricle. The child underwent stenting of the patent ductus arteriosus and balloon atrial septostomy on the 11th day of life. Pulmonary artery banding was done at the age of 1 month. During the current admission, a computerized tomography scan revealed the complex connections of the great vessels (Fig 1). A 4-chamber transesophageal echocardiography view displayed the morphologic left atrium, which was on the right side, and multiple coronary artery fistulae opening into an enlarged chamber located superiorly (Fig 2; Video 1). The atrial septal defect could be visualized in the bicaval view (Fig 3; Video 2). A modified midesophageal 4-chamber view displayed a large superior chamber, as well as the atrial septal defect (Fig 4). Agitated saline was injected into a right upper extremity vein. The agitated saline appeared first in the right internal jugular vein and then appeared in the upper atrial chamber, right side atrium (morphologic left atrium), followed by an appearance in the right ventricle (Fig 5, A-D; Video 3). Slight retraction of the probe from the 4-chamber view displayed a right superior vena cava on the right side that was moving into close juxtaposition with the morphologic left atrium (Fig 6; Video 4). Correlation with computerized tomography findings revealed the identity of the structure seen by transesophageal echocardiography into which the right superior vena cava and the coronary artery fistulae were draining. What is the diagnosis? Fig. 2(A) Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with (B) color Doppler displaying an enlarged chamber (white query sign) with multiple coronary artery fistulae opening into the chamber. mLA, morphologic left atrium; RV, right ventricle; TV, tricuspid valve View Large Image Figure Viewer Download Hi-res image Fig. 3Bicaval midesophageal transesophageal echocardiography view displaying the atrial septal defect. View Large Image Figure Viewer Download Hi-res image Fig. 4Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with color-flow Doppler displaying the atrial septal defect and a large upper chamber marked by white query sign. View Large Image Figure Viewer Download Hi-res image Fig. 5Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying (A) the agitated saline echo contrast injected into an upper limb vein entering the right superior vena cava, (B) the coronary sinus and the morphologic right atrium on the right side, (C) the morphologic left atrium on the right side, and (D) the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 6Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying the right superior vena cava and morphologic right atrium in close proximity. ASD, atrial septal defect; LAA, morphologic left atrial appendage; mLeft atrium, morphologic left atrium; mR, morphologic right atrium; RSVC, right superior vena cava; RV, right ventricle; TV, tricuspid valve. View Large Image Figure Viewer Download Hi-res image

Multiple Color Doppler Flow Jets Into The Atria in a Child With Heterotaxy Syndrome

A nine-month-old girl (weight: 7.92 kg; height: 70 cm) was taken to the operating room for the creation of a bilateral bidirectional Glenn shunt and atrial septectomy. Patent ductus arteriosus stenting had been done when the child was four days old. Transthoracic echocardiography showed heterotaxy (left atrial isomerism) with situs inversus, discordant atrioventricular and concordant ventriculoarterial connections (ventricular ‘noninversion’), a large ventricular septal defect, membranous pulmonary atresia with a hypoplastic main pulmonary artery, confluent branch pulmonary arteries, aorta anterior and to the right of the pulmonary artery, and bilateral superior vena cava with no bridging vein. The left superior vena cava was draining into the left-sided atrium (morphologic right atrium), while the right superior vena cava was draining into the same atrium via a massively dilated coronary sinus. The inferior vena cava was interrupted, with hemzygous continuation to the left superior vena cava. A computerized tomography scan revealed a right-sided stomach with a midline liver, and demonstrated the complex vascular communications (Fig 1, A and B). Transesophageal echocardiography confirmed the preoperative diagnosis (Fig. 2, Fig. 3; Videos 1 and 2). Color- flow Doppler mapping in the mid-esophageal view demonstrated multiple jets into both the atria and the right-sided ventricle (Fig 4, A and B; Videos 3 and 4). Pulse-wave Doppler of a dominant jet revealed a biphasic flow (Fig 5; Video 5). The intramyocardial origin of the jets was traced toward the aorta (Fig 6; Video 6). Fig. 2The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map showing demonstrating the anatomic abnormality in the child. View Large Image Figure Viewer Download Hi-res image Fig. 3The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map demonstrating the left superior vena cava. Left SVC, left superior vena cava. View Large Image Figure Viewer Download Hi-res image Fig. 4(A) The midesophageal transesophageal echocardiography views with color-flow Doppler map demonstrating jets (white arrows) into the left atrium. (B) The right atrium and the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 5The midesophageal pulse-wave Doppler signal interrogation of the jet by transesophageal echocardiography. View Large Image Figure Viewer Download Hi-res image Fig. 6The midesophageal transesophageal echocardiography view displaying the color-flow Doppler mapping delineating the origin of the jet (white arrows). View Large Image Figure Viewer Download Hi-res image

Neonatal intra-atrial baffle repair for isolated ventricular inversion with left isomerism: a case report

BackgroundDiscordant atrioventricular connection with concordant ventriculoarterial connection, otherwise known as isolated ventricular inversion (IVI), is an extremely rare congenital cardiac malformation. Reports on the corrective surgery for this anomaly in neonates are few, and the procedure is difficult and complicated. Herein, we report our use of atrial septostomy as a palliative procedure followed by corrective surgery for the repair of neonatal IVI with situs ambiguous(inversus) morphology.Case presentationA 2-day-old girl weighing 3.5 kg was admitted to our hospital with a low oxygen saturation (SpO2) of 70% She was diagnosed with IVI [situs ambiguous(inversus), D-loop, and D-Spiral], atrial septal defect, patent ductus arteriosus (PDA), interrupted inferior vena cava with azygos continuation to the left superior vena cava (SVC), and polysplenia by transthoracic echocardiography and cardiac computed tomography. We planned to perform corrective surgery and decided to first increase interatrial mixing by performing surgical atrial septostomy and PDA ligation 7 days after birth. However, despite the surgical septostomy, pulmonary venous blood flowed toward the right ventricle via the tricuspid valve rather than toward the left-sided atrium and hypoxemia persisted. We decided to perform the intra-atrial switch procedure at the age of 17 days via a re-median sternotomy. The cardiopulmonary bypass (CPB) circuit was established with ascending aorta and venous drainage through the SVC and hepatic veins. Utilizing a left-sided atrium(l-A) approach, a bovine pericardial patch was used for the intra-atrial baffle, which was trimmed into a trouser-shaped patch. Continuous suture using the patch was lying from the front of the right-sided upper pulmonary vein and rerouted SVC, hepatic vein, and coronary sinus to the tricuspid valve. Overall, CPB weaning proceeded smoothly; however, direct current cardioversion was performed for junctional ectopic tachycardia. The postoperative course was uneventful. Her postoperative SpO2 improved (approximately 99–100%); overall, the patient showed clinical improvement. Discharge echocardiography showed normal biventricular function and an intact atrial baffle with no venoatrial or atrioventricular obstruction.ConclusionWe successfully performed an intra-atrial switch procedure for isolated ventricular inversion in a neonate. Long-term follow-up will be necessary to ensure the maintenance of optimal cardiac function.

Successful balloon valvuloplasty of a subpulmonic membrane associated with cor triatriatum dexter: a case report

BackgroundSubpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare. Association with cor triatriatum dexter and success of subpulmonic balloon valvuloplasty have never been reported, at least to the best of our knowledge.Case presentationA 3-year-old Moroccan boy was referred to our tertiary care hospital with complaints of dyspnea on moderate exertion. A physical examination revealed parasternal lift, systolic thrill, and a 4/6 ejection systolic murmur, best heard over the left second intercostal space. His oxygen saturation was 99% on room air. Two-dimensional echocardiography showed a discrete circumferential membrane just below the pulmonic valve and a right atrial membrane. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 85 mmHg across the subpulmonic membrane and no significant gradient across the right atrial membrane. Balloon dilation of the subpulmonic membrane was performed and the pressure gradient came down to 50 mmHg. During follow-up, he reported marked improvement in terms of exercise tolerance. Transthoracic echocardiography showed residual pressure gradient of approximately 40 mmHg across the membrane. Surgery resection of the two membranes was programmed, but he died after an extracardiac disease (appendicular peritonitis).ConclusionsSubpulmonic membrane as an isolated cause of right ventricular outflow tract obstruction is rare. Its association with cor triatriatum dexter is even less common. The result of percutaneous balloon valvuloplasty of subpulmonic membrane is an interesting alternative while waiting for surgery. Surgery is currently the preferred modality of treatment with the resection of both right atrial and subpulmonic membranes.

A new anatomic approach of the ventricular septal defect in the interruption of the aortic arch.

The aim of this study was to analyse the anatomy of the ventricular septal defect (VSD) in heart specimens with interruption of the aortic arch (IAA) in order to explore the hypothesis of different embryologic mechanisms for the different anatomic types of IAA. We examined 42 human heart specimens, 25 with IAA as the main disease with concordant atrioventricular and ventriculo-arterial connections and two distinct great arteries, and 17 hearts with IAA associated with other malformations [six common arterial trunk (CAT), five double-outlet right ventricle (DORV), three transposition of the great arteries (TGA), three atrioventricular septal defect (AVSD)]. The interruption was classified according to Celoria and Patton. We focused on the anatomy of the VSD viewed from the right ventricular side. There were 15 IAA type A, 27 type B, no type C. The VSD in IAA type B was always an outlet VSD, located between the two limbs of the septal band, with posterior malalignment of the outlet septum in hearts with concordant ventriculo-arterial connections, without any fibrous tricuspid-aortic continuity. In addition, the aortic arch was always completely absent. Conversely, the VSD in IAA type A could be of any type (outlet in six, muscular in four, central perimembranous in two, inlet in three) and the aortic arch was either atretic or absent. In addition, IAA type B, when found in the setting of another anomaly, was always associated with neural crest-related anomalies (CAT and DORV), whereas IAA type A was found in association with anomalies not related to the neural crest (TGA and AVSD). These results reinforce the hypothesis that different pathogenic mechanisms are responsible for the two types of IAA, and the inclusion of IAA type B in the group of neural crest defects. Conversely, IAA type A could be due to overlapping mechanisms: flow-related defect (coarctation-like) and neural crest contribution.

D-Transposition of the Great Arteries: A New Era in Cardiology

Before the 1950s, D-transposition of the great arteries was associated with nearly 90% mortality within the first year of life. The Mustard and Senning procedures resulted in a significant increase in the lifespan of these patients but with notable long-term complications, including arrhythmias, sinus node dysfunction, chronotropic incompetence, and right ventricular systolic dysfunction. The arterial switch operation (first described by Adib Jatene) initially resulted in nearly universal death. However, the use of coronary buttons for coronary artery translocation has improved operative survival dramatically. It is now considered the treatment of choice in patients amendable to the arterial switch operation. Considered an anatomic repair, resulting in concordant ventriculoarterial connections and a systemic left ventricle, the arterial switch operation reduces the incidence of ventricular dysfunction. However, it is also associated with long-term complications, including aortic root dilatation, aortic valve regurgitation, right ventricular outflow tract obstructions, coronary artery stenosis/compression, and branch pulmonary artery stenosis.

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

Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach. To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up. A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections. 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.

Clarifying the anatomy of hearts with concordant ventriculo-arterial connections but abnormally related arterial trunks.

Hearts in which the arterial trunks arise from the morphologically appropriate ventricles, but in a parallel manner, rather than the usual spiralling arrangement, have long fascinated anatomists. These rare entities, for quite some time, were considered embryological impossibilities, but ongoing experience has shown that they can be found in various segmental combinations. Problems still exist about how best to describe them, as the different variants are often described with esoteric terms, such as anatomically corrected malposition or isolated ventricular inversion. In this review, based on our combined clinical and morphological experience, we demonstrate that the essential feature of all hearts described in this manner is a parallel arrangement of the arterial trunks as they exit from the ventricular mass. We show that the relationship of the arterial roots needs to be described in terms of the underlying ventricular topology, rather than according to the arrangement of the atrial chambers. We then discuss the importance of determining atrial arrangement on the basis of the morphology of the appendages, following the precepts as set out in the so-called "morphological method" and distinguished according to the extent of the pectinate muscles relative to the atrioventricular junctions as opposed to basing diagnosis on the venoatrial connections. We show that, when approached in this manner, the various combinations can be readily diagnosed in the clinical setting and described in straightforward way.

Tricuspid Atresia 18 Years Post Glenn: Is Fontan Necessary in All Cases?

Tricuspid atresia is a rare cyanotic congenital heart disease, first described by Kreysig (1817), has an incidence of 1% to 5%. It is incompatible with longevity without surgical intervention. Glenn (1958) was successful in using a superior vena cava to right pulmonary artery anastomosis. Fontan (1958) was successful in separating the right and left cardiac circulation; this is the surgical method of choice, with death of 17% by 20 years of age. The Fontan circulation has burdens of multisystem abnormalities, including ventricular dysfunction (72%), dysrhythmias (40%) and renal, hepatic, gastrointestinal and pulmonary complications. The index case is the oldest documented report of tricuspid atresia with rudimentary right ventricle and concordant ventriculo-arterial connections, 18 years post Glen only, from two years of age. She is asymptomatic, of high intellect, never used medications and is now functioning at New York Heart Association (NYHA) I-II, at 20 years of age. Her survival and function was secondary to an innate large atrial and ventricular communication with normal left ventricle, mitral, aortic and pulmonary valves, indicating Fontan may not be needed in all structural anatomic cases of tricuspid atresia.

Holes and channels between the ventricles revisited.

Although holes, or channels, between the ventricles are the commonest congenital cardiac malformations, there is still no consensus as to how they can best be described and categorised. So as to assess whether it is possible to produce a potentially universally acceptable system, we have analysed the hearts categorised as having ventricular septal defects in a large archive held at Birmingham Children's Hospital. Materials and methods We analysed all the hearts categorised as having isolated ventricular septal defects, or those associated with aortic coarctation or interruption in the setting of concordant ventriculo-arterial connections, in the archive of autopsied hearts held at Birmingham Children's Hospital, United Kingdom. We found 147 hearts within the archive fulfilling our criterions for inclusion. All could be classified within one of three groups depending on their borders as seen from the right ventricle. To provide full description, however, it was also necessary to take account of the way the defects opened to the right ventricle, and the presence or absence of alignment between the septal components. By combining information on the phenotypic specificity defined on the basis of their borders, the direction of opening into the right ventricle, and the presence or absence of septal malalignment, it proved possible to categorise all hearts examined within the archive of Birmingham Children's Hospital. Our findings have necessitated creation of new numbers within the European Paediatric Cardiac Code.

Discrete subpulmonic membrane in association with isolated severe pulmonary valvar stenosis

BackgroundSubpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare.Case presentationA 7 – year – old boy was referred to a tertiary care hospital with complaints of dyspnea on moderate exertion and palpitations of about 2 years duration. Physical examination revealed parasternal lift, systolic thrill and a 4/6 ejection systolic murmur, best heard over the left 2nd intercostal space. His oxygen saturation was 88% on room air. Two-dimensional echocardiography showed a thickened pulmonary valve with fused leaflets that show severe systolic doming. There was a discrete subpulmonic membrane about 1.3 cm below the pulmonary valve annulus. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 185 mmHg across the pulmonary valve. Balloon dilation of the pulmonary valve was performed and the pressure gradient came down to 50 mmHg. Follow-up transthoracic echocardiography showed residual pressure gradient of about 50 – 60 mmHg across the pulmonary valve. The residual pressure gradient appeared to be mainly subvalvar, as seen on the continuous wave Doppler tracing. The patient reported marked improvement in terms of exercise tolerance and subjective symptoms.ConclusionsAssociation of subpulmonic membrane with severe pulmonary valvar stenosis, concordant ventriculoarterial connection and intact ventricular septum is rare. When it occurs, the result of percutaneous valve dilation may be suboptimal.

How Best Can We Define Double Outlet Right Ventricle When Describing Congenitally Malformed Hearts?

How Best Can We Define Double Outlet Right Ventricle When Describing Congenitally Malformed Hearts?

Concordant Ventriculoarterial Connections With Parallel Arterial Trunks, Divided Left Atrium, and Juxtaposed Atrial Appendages

We describe a patient with concordant ventriculoarterial connections with parallel arterial trunks, divided left atrium (cor triatriatum sinister), ventricular septal defect, bilateral superior caval veins, and juxtaposed atrial appendages. The aorta was anterior and left sided. We discuss the morphological features of this rare condition, and the diagnostic dilemma it produced.

Anatomy of Discordant Atrioventricular Connections

The term discordant atrioventricular connections refers to the situation in which the ventricles are connected inappropriately to the atrial chambers. In most instances, the connections of the great arteries are also abnormal, with the aorta and the pulmonary trunk arising from morphologically inappropriate ventricles. This combination results in the presence of so-called congenitally corrected transposition. Double-outlet right ventricle is occasionally present, while concordant ventriculoarterial connections may be seen rarely. Most such hearts have a range of additional abnormalities, including ventricular septal defects; outflow tract obstruction, usually of the morphologically left ventricle; anomalies of the morphologically tricuspid valve; and a highly abnormal location of the specialized atrioventricular conduction axis. Some examples exhibit bizarre abnormalities of ventricular relationships and topology, including criss-cross atrioventricular connections and superoinferior ventricular relations. In describing the anatomy of these malformations, it is important to use a step-by-step segmental approach to the documentation of the connections and associated defects in each case and to avoid potentially confusing shorthand terms.

Double outlet right atrium with coexisting double inlet left ventricle and concordant ventriculoarterial connections: a fascinating variant of the Holmes heart

Occasionally complex congenital cardiac disease presents for the first time in adulthood, encouraging us to reflect anew on our understanding of familiar conditions. A 59-year-old woman, who had had two normal pregnancies, attended with breathlessness and was found to have a double inlet left ventricle with concordant ventriculoarterial connections and a straddling tricuspid valve adherent to the malaligned ventricular septum.

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome

To evaluate the intrauterine course and outcome of tricuspid atresia detected in the fetus. This was a retrospective review of all confirmed cases of tricuspid atresia detected prenatally between 1998 and 2006 in three tertiary referral centers in Germany. Fifty-four cases of tricuspid atresia were detected prenatally during the study period and confirmed postnatally: 28 (51.9%) cases had a concordant ventriculoarterial connection of which 14 also had pulmonary outflow obstruction; 25 (46.3%) cases had a discordant ventriculoarterial connection of which 14 also had aortic outflow obstruction, six had pulmonary outflow tract obstruction and two had other associated intracardiac anomalies; and one (1.9%) had a common arterial trunk. The peak velocity index for veins in the ductus venosus was significantly elevated in 19 of the 37 (51.4%) cases assessed; however, this finding did not correlate with adverse intrauterine outcome. There were associated extracardiac anomalies in 12 cases: five with chromosomal anomalies, two with VACTERL association, one with unilateral renal agenesis, one with hypospadia, one with hydrothorax, one with megacystis and one with agenesis of the ductus venosus. Seventeen of the 54 (31.5%) cases underwent termination of pregnancy, two (3.7%) died in utero, two (3.7%) died in infancy and 33 (61.1%) children survived with a median follow-up of 26 (range, 12-120) months. Prenatal echocardiography correctly anticipated the postnatal course and the need for neonatal intervention in 29/35 (82.9%) continued pregnancies; in the remaining six (17.1%) cases the right outflow tract obstruction had been underestimated. Tricuspid atresia and the frequently associated intracardiac anomalies can be diagnosed in the fetus with considerable accuracy. A thorough search for extracardiac malformations should be performed in order to rule out chromosomal anomalies and multiple malformation syndromes. Elevated pulsatility in the ductus venosus does not indicate cardiac failure. The short-term overall survival in continued pregnancies in our study exceeded 89%, with the greatest rate of loss being in the first year of postnatal life.

Catheter closure of an atrial septal defect in anatomically corrected malposition with left juxtaposition of atrial appendages

Anatomically corrected malposition is characterized by concordant atrioventricular and ventriculoarterial connection and parallel arrangement of arterial trunks. Left juxtaposition of the atrial appendages, a frequent association of this anomaly, alters the atrial septal plane. In this report, I describe the insertion of a device to close an atrial septal defect, as guided by transoesophageal echocardiography.

Glenn bidirecional com cianose precoce por conexão veno-venosa pela veia hemiázigo

CLINICAL DATA6 year-five-month-girl, 15 kg, from Comodoro, MT. Full-term patient, presented fatigue and cyanosis presentationat 3 months of life and underwent banding of the pulmonarytrunk (BPT) through left thoracotomy in another Service at8 months. The mother reports that after surgery there wasan improvement of dyspnea, but not of cyanosis and weightgain.Due to difficulties found by the patient at the UnifiedHealth System (SUS) [1], she was referred to our Servicewith 3 years and 6 months of life. Thus, tricuspid atresiawith concordant ventriculoarterial connection, restrictiveinterventricular communication, foramen ovale interatrialcommunication and effective BPT were diagnosed.Following our protocol for referring patients withuniventricular physiology in different surgical stages (Glennand Fontan), bidirectional Glenn operation (superiorbidirectional cavopulmonary) was performed withconnection between the superior vena cava (SVC) and rightpulmonary artery (RPA), ligation and section of the azygousvein, resection of the interatrial septum and new and moretight BPT, allowing small anterograde flow and a pulmonaryartery pressure less than 15 mmHg. The patient wasdischarged from hospital on the seventh postoperative dayusing aspirin and with peripheral saturation of 92%.After 1 year and 6 months, the mother reported againimportant patient’s cyanotic episodes. At the time, theperipheral saturation at rest was 84%, with indication ofechocardiography with injection of microbubbles fordetection of arteriovenous or venovenous connections thatcould cause such very severe unsaturation. Contrast inright upper limb (RUL) was injected, which moved throughthe right atrium (RA) from the inferior vena cava (IVC),suggesting the presence of flow between the innominatevein and IVC. The connection of the SVC to RPA inbidirectional Glenn operation presented excellent flow andthe tight BPT was effective.Thus, hemodynamic study was indicated for accurateassessment of the anatomy and pressure in the pulmonaryartery, but due to personal and Brazilian geographicaldifficulties, the mother returned only after almost two years,when the child presented a significant worsening ofcyanosis and peripheral saturation of 76% at rest.On physical examination the patient presented in goodgeneral condition, afebrile, eupneic and cyanotic +++/4+.Normal

Invited Commentary

Invited Commentary