Aorto-ventricular tunnel with three orifices: a unique case report diagnosed by transthoracic echocardiography.

BACKGROUND: Aorto-ventricular tunnel (AVT) is an abnormal communication channel between the ascending aorta and the ventricle. It commonly has two orifices, i.e., one aortic opening and one ventricular opening. In this study, we present a unique case of AVT with three orifices: one aortic opening, one LV opening, and one RV opening. CASE PRESENTATION: A 64-year-old male presented with chest discomfort and dyspnea on exertion lasting the past six months. Physical examination revealed a grade 4/6 continuous biphasic murmur along the left sternal edge and a grade 3/6 systolic murmur at the apex. Transthoracic echocardiography (TTE) demonstrated: (1) an AVT with three orifices, i.e., one aortic opening, one LV opening, and one RV opening. The LV and RV openings were located in the left and right ventricular outflow tracts, respectively. (2) The aortic valve (AV) was calcified with a small aneurysm at the non-coronary cusp. (3)The mitral valve (MV) chordal rupture of the P2 and P3 segments was observed in the posterior leaflet with severe eccentric regurgitation. Subsequent coronary computed tomography angiography (CTA) further confirmed the diagnosis of AVT with three openings, and clarified the coronary arteries normally arose from the aortic sinuses. The patient was then referred for surgical treatment, consisting of closure of three AVT orifices, AV replacement, and MV replacement. Six months following surgery, the patient was asymptomatic. TTE demonstrated normal mechanic AV and MV function, and there was no residual shunt among the ascending aorta, LV and RV. CONCLUSIONS: It is the first case to report an AVT with three orifices. This paper described the entire process from diagnosis to treatment of this unique case, thus providing some novel insights into AVT.

Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.

Abnormal congenital aorto-cardiac communications (CACC) are a heterogeneous constellation of anomalies that provide an abnormal connection between the aorta and other cardiac chambers or structures, including the atria, ventricles, the main pulmonary artery, and the coronary sinus. The current terminology of CACC has significant errors and shortcomings including inconsistent and interchangeable use of terms of fistula and tunnel and lack of an inclusive classification with practical information on therapeutic management. The aims of this study were threefold: firstly, to perform a concise narrative review of congenital pathologic connections between the aortic root and cardiac chambers which include rupture of congenital sinus of Valsalva aneurysm, aorto-left ventricular and less commonly right ventricular tunnels, coronary cameral fistulas, and aorto-atrial communications; secondly, to investigate the differentiating features of the so-called aorta right atrial tunnel (ARAT), with and without coronary artery take-off from the tunnel, and coronary cameral fistula (CCF) by applying a differential diagnostic assistance toolbox to two groups of patients with ARAT and CCF; and lastly, to propose a practical and inclusive anatomic-therapeutic classification for CACCs. The two main cornerstones of the proposed classification are the type of the connector between the aorta and cardiac chamber (hole versus passage) and the nature of the connecting passage ( anatomic versus extra-anatomic). We classified CACCs into three types. Depending on the intramural versus extramural course of the extra-anatomic connecting passage, type 3 is further subdivided into type 3A and type 3B.

A Review of the Surgical Management of Aorto-ventricular Tunnels.

We present a synthesis of 95 published investigations of the exceedingly rare tunnels that can exist between the aortic root and the left or right ventricles. From the 220 suitable cases included in these investigations, we reviewed the clinical presentations, modalities used for diagnosis, surgical approaches, and outcomes. Diagnostic information was provided by clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, magnetic resonance imaging, cardiac catheterization, and angiocardiography. These techniques elucidated the coronary arterial origins and associated defects and defined the disease before surgery. Patients occasionally present with an asymptomatic cardiac murmur and cardiomegaly, but most suffer cardiac failure in the first year of life when the tunnel enters the left ventricle. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks of gestation. Associated defects, involving the proximal coronary arteries or the aortic or pulmonary valves, are present in nearly half the cases. Prompt diagnosis and surgical repair are important for a favorable outcome. Overall, operative mortality has been cited to be between 3% and 8.3%. Associated congenital coronary arterial anomalies, residual severe aortic stenosis, poor left ventricular function, and rupture of an infected suture line have been the reported causes of death. Despite early surgical intervention, an incidence of 16% to 60% postoperative residual aortic regurgitation of varying severity has been reported. The requirement of further repair or replacement of the aortic valve ranges from 0% to 50%. We submit that an increased appreciation of these details relative to the tunnels will contribute to improved surgical management.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.

Aorto-Right Ventricular Tunnel in Transposition of the Great Arteries.

Aorto-ventricular tunnel is an extremely rare congenital heart defect, consisting of failure of attachment of an aortic leaflet along the semilunar hinge. In all published reports the leaflet involved was either the right coronary leaflet, most frequently, or the left coronary leaflet, in most of the cases opening toward the left ventricle, with only one-eighth of the reported cases communicating with the right ventricle. Treatment of the aorto-ventricular tunnel has been anecdotally reported by interventional closure with a device and more frequently with surgical approach, either as an isolated malformation or as associated lesions. To the best of our knowledge, the presence of an aorto-ventricular tunnel of the non-adjacent aortic leaflet in transposition of the great arteries has never been reported. We have observed an aorto-ventricular tunnel involving the non-adjacent leaflet of the aortic root, which after arterial switch became the pulmonary root. The patient presented 18 years after the arterial switch with progressive dilatation of the right ventricle due to severe degree of pulmonary valve regurgitation, confirmed by echocardiography and cardiac MRI. Indication for surgery was given with the plan for a pulmonary valve implantation. Because of the intra-operative finding of disconnection of the anterior leaflet of the pulmonary valve (former aortic valve) along the semilunar hinge, the surgical plan was modified and the anterior leaflet was attached to the valve annulus, with subsequent plasty in correspondence with the right and left commissurae to reduce the size of the dilated annulus to normal diameter. The post-operative course was uneventful, with extubation after few hours and discharge 4 days after surgery, with echocardiography showing trivial degree of pulmonary valve regurgitation. The patient remains in good conditions 6 months after surgery.

Repair of an aorto-right ventricular tunnel in a newborn.

A newborn presented with an aorto-right ventricular tunnel, a defect connecting the left aortic sinus to the right ventricle. The patient underwent repair on 4th day of life.

Túnel aórtico a ventrículo izquierdo Reporte de caso y manejo anestésico / Computed tomography as a diagnostic guidance for malignant pleural mesothelioma

Resumen: El hospital del Instituto Mexicano del Seguro Social (IMSS), Centro Médico Nacional (CMN) La Raza, "Dr. Gaudencio González Garza" es referente en la atención médica de nuestro país. En él se atiende preferentemente a población pediátrica y, de ésta, a un gran porcentaje con patología cardiaca congénita. La incidencia de afecciones en este hospital concuerda con lo reportado en la literatura, siendo las más frecuentes: comunicación interventricular (CIV), comunicación interauricular (CIA), persistencia de conducto arterioso (PCA), coartación aórtica (CoAo), tetralogía de Fallot, conexión anómala de venas pulmonares (CAVP), y transposición de las grandes arterias (TGA), entre otras. El túnel aórtoventricular a es una cardiopatía congénita poco frecuente, ya que ocupa < 0.1% de todas las malformaciones congénitas; el 90% de los casos comunica al ventrículo izquierdo. Hasta el 2007 se han reportado cerca de 130 casos en la literatura, con predominio sobre el sexo masculino en relación 2:1. Algunos pacientes presentan soplo en el corazón y cardiomegalia, pero la mayoría sufren de insuficiencia cardiaca en el primer año de vida. El estudio de elección para el diagnóstico es la ecocardiografía. Por lo general se tratan quirúrgicamente, pero en casos seleccionados se puede cerrar mediante cateterismo percutáneo. En nuestro hospital, este caso es el segundo en quince años, ambos en varones y tratados en forma quirúrgica. Inicialmente, el diagnóstico se sospechó por ecocardiografía y se realizó en forma definitiva mediante angiotomografía. La información respecto al manejo anestésico para esta cardiopatía congénita es escasa, Carol L. Lake y cols., describen en forma breve los objetivos anestésicos, mejorando la fracción de eyección y la poscarga, así como la disminución de las resistencias vasculares sistémicas; el mejorar el mayor flujo hacia delante; la terapia crónica con vasodilatadores, diuréticos o digital; el uso de halogenados, y las medidas previas a la canulación cardiaca y la salida del by-pass. Debido a la escasa información sobre el manejo anestésico aunada a la poca frecuencia de esta malformación, nos dimos a la tarea de realizar el reporte de este caso para dar herramientas en el tratamiento desde el punto de vista anestésico.

Abstract: The hospital of the Mexican Institute of Social Security (MISS), the National Medical Center La Raza "Dr. Gaudencio Gonzalez Garza" (NMC La Raza), is a center of reference and concentration for medical attention provided in our country, preferably we provide pediatric medical care and a high percentage of those with congenital cardiac pathology. The incidence in our hospital is according to the results posted in the medical journals, being the most frecuent: Interventricular Communication (IVC), Interatrial Communication (IAC), Patent Ductus Arteriosus (PDA), Coarctation of the Aorta (CoA), tetralogy of Fallot, Total Anomalous Pulmonary Venous Connection (TAPVC), Transposition of the Great Arteries (TGA), among others. The aorto-ventricular tunnel is an infrequent congenital heart disease less than 0.1% of all congenital malformations, of which 90% of the cases communicate the left ventricule. About 130 cases have been reported until 2007, prevailing the male cases in ratio 2:1. Some patients present a heart murmur and cardiomegaly, but most of them suffer from heart failure in the first year, the study of choice for diagnosis is echocardiography. Generaly they are treated with surgery, but in selected cases they can be treated with percutaneous catheterization. In our hospital this is the second one in 15 years, both male and they were treated with surgery. The diagnosis was initially suspected by echocardiography and through tomography was performed in final form. The information in reference to the anesthetic management for this congenital heart disease is limited, Carol L. Lake et al., described briefly the anethetics objetctives improving the ejection fraction and the afterload; the decrease of the systemic vascular resistance and improving more flow forward, as well as chronic vasodilator therapy, diuretics or digital; the use of halogenated and the previous mesurements to the cardiac cannulation, and to the bypass output. Due to the insufficient information about the anesthetic management coupled with the rarity of this malformation, we took on the task of reporting this case providing tools for treatment from the point of view anesthetic.

Surgical repair of aorto-ventricular tunnel.

This report presents a trans-aortic trans-infundibular double-patch repair of an aorto-left ventricular tunnel in a 17-day old male child. It reviews the literature on aorto-ventricular tunnels and debates strategic and surgical options available to correct these defects. Diagnosis of the tunnel in the case described herein was made prenatally. The child was presented with left ventricular dilatation with early and progressive signs of congestive heart failure. Decision for early surgical correction was made considering the quantum of regurgitant jet, diastolic backflow in the aorta and consequent left ventricular dilatation. After establishing cardiopulmonary bypass and arresting the heart, the aorta was transversely opened above the sinotubular junction and the tunnel identified. Aortic valve morphology and the coronary ostia not involved in the tunnel were carefully investigated. Through a transverse infundibulotomy, the thinned outer wall of the tunnel abutting the posterior wall of the infundibular septum was slit open vertically, thus clearly defining the aortic and the left ventricular orifices. The distal tunnel orifice, now presenting as a subaortic Ventricular septal defect (VSD), was closed using a xenopericardial patch and running polypropylene 7-0 stitches. The aortic end of the tunnel was closed in a similar fashion through the aortotomy, remaining clear of the right coronary artery and respecting the aortic valve geometry. The marsupialized wall of the tunnel as seen through the infundibulotomy was sutured with polypropylene stitches. The aortotomy and right ventriculotomy were closed. The postoperative course was uneventful. A follow-up echocardiography at 2 years showed a perfect outcome with no residual tunnel, no aortic stenosis and trivial aortic regurgitation.