Multimodality imaging for comprehensive non-invasive diagnosis of aorto-left ventricular tunnel in infancy.

BACKGROUND: Aorto-left ventricular tunnel (ALVT) is a paravalvular communication between aorta and left ventricle. It is one of the rare congenital heart diseases which could present with heart failure. CASE PRESENTATION: A case of ALVT was diagnosed in infancy. Preliminary assessment was possible using conventional echocardiography; however, functional assessment and accurate anatomy of ALVT were further verified via variable imaging modalities starting from speckle tracking and three-dimensional echocardiography to cardiac CT angiogram. The tunnel was successfully repaired with uneventful recovery. CONCLUSIONS: Multimodality imaging can accurately assess cardiac function and demonstrate the anatomy of ALVT noninvasively to plan for successful intervention.

Aorto-left ventricular tunnel: the necessity of early surgical intervention.

Aorto-left ventricular tunnel, also known as aorto-left ventricular fistula, is a rare CHD characterised by an abnormal connection between the aorta and the left ventricle. This article presents a comprehensive case report on aorto-left ventricular tunnel, encompassing clinical presentation, diagnostic approaches, treatment options, and outcomes, with a specific focus on the necessity of early surgical intervention.

Diagnosis of an aortico-left ventricular tunnel in a fetus: a case report.

An aortico-left ventricular tunnel is a rare congenital heart disease, and its prenatal diagnosis is even rarer. This report describes a fetus diagnosed with an aortico-left ventricular tunnel at 26 weeks of gestation. After delivery, the infant exhibited cyanosis and cessation of breathing. After resuscitation, he was transferred to the neonatal intensive care unit. Echocardiography confirmed an aortico-left ventricular tunnel. The infant survived after surgical repair. An aortico-left ventricular tunnel can be diagnosed by antenatal ultrasound, and prompt neonatal management can help to prevent perinatal morbidity and mortality.

Echocardiographic diagnosis of aorto-left ventricular tunnel with supravalvular pulmonic stenosis in a Shih-tzu dog.

Background: The aorto-left ventricular tunnel (ALVT) is a congenital extracardiac channel that connects the ascending aorta to the left ventricle. Case Description: A 2-year-old Shih-tzu dog presented with mild exercise intolerance. Echocardiography revealed an abnormal slit-like tunnel structure connecting the ascending aorta to the left ventricle, with diastolic blood flow from the aorta to the left ventricle. Echogenic membranous stenosis was observed in the main pulmonary artery. Based on these findings, the dog was diagnosed with ALVT and type I supravalvular pulmonic stenosis. Conclusion: This is the first case report of ALVT in veterinary medicine that describes diagnostic imaging findings. ALVT should be considered in dogs with an aortic regurgitation murmur and can be detected by echocardiography.

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.

Aorto-Left Ventricular Tunnel - Prenatal Diagnosis and Outcome.

PURPOSE: Aorto-left ventricular tunnel (ALVT) is an extremely rare, albeit prenatally detectable, extracardiac channel that connects the ascending aorta to the cavity of the left ventricle. MATERIALS AND METHODS: All ALVTs diagnosed prenatally (2006-2020) in five tertiary referral centers were retrospectively assessed for prenatal ultrasound findings, intrauterine course, postnatal outcome, and surgical treatment. We focused on the size of the tunnel and alterations of perfusion of the left ventricular outflow tract and aortic arch. RESULTS: 11 fetuses were diagnosed with ALVT at a mean gestational age of 24.8 weeks. All cases were associated with severe dilatation of the left ventricle and a to-and-fro flow in the left outflow tract. Signs of congestive heart failure were present in five fetuses, four of which were terminated and one of which died in the neonatal period. One fetus died in utero at 34 weeks without prior signs of cardiac failure. Of the five survivors, two underwent the Ross procedure. In both cases the prenatal left ventricular outflow was exclusively via a large tunnel. The remaining three neonates underwent patch closure of the tunnel. In these cases, the prenatal outflow of the left ventricle was via the aortic valve and simultaneously over the tunnel. CONCLUSION: Prenatal diagnosis of ALVT should be considered in the presence of left ventricular hypertrophy, dilatation of the aortic root, and to-and-fro flow in the aortic outflow tract. Signs of heart failure are associated with an unfavorable outcome. Large tunnels, particularly in combination with the absence of flow over the aortic valve, may be an unfavorable predictor of surgical repair.

Successful percutaneous closure of a recurrent aorto-left ventricular tunnel with an Amplatzer Vascular Plug II.

Aorto-left ventricular tunnel is an extremely rare CHD clinically impressing as aortic regurgitation. This is usually corrected surgically, sometimes by percutaneous catheter intervention. Recurrent aorto-left ventricular tunnel has been reported occasionally. Reports of percutaneous closure of such recurrent aorto-left ventricular tunnels are rare. We hereby describe successful closure of a recurrent aorto-left ventricular tunnel with an Amplatzer Vascular Plug II.

The aorto-left ventricular tunnel from a fetal perspective: Original case series and literature review.

INTRODUCTION: Aorto-left ventricular tunnel (ALVT) accounts for <0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT. METHODS: All members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow-up. Additionally, a systematic search of the literature was performed. RESULTS: Twenty fetuses with ALVT were identified in 10 participating centers (2001-2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac-thorax ratio (95%), left ventricular end-diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21 + 6 weeks (range, 19-24): all showed signs of hydrops prior to 24 weeks or at autopsy. All others (60%, 12/2) were live-born (median GA 38 + 4, range 37-40), underwent surgery and were alive at last follow up (median 3.2 years, range 0.1-17). The literature reported 22 ALVT fetuses with similar outcome. CONCLUSIONS: In the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24 weeks without hydrops are likely to have a good outcome.

Surgical repair of an aortico-left ventricular tunnel with Takayasu's arteritis.

Aortico-left ventricular tunnel is a very rare congenital cardiac anomaly, always arises from the right coronary sinus and enters the left ventricle. However, aortico-left ventricular tunnel associated with Takayasu's arteritis has not been described so far in the literature. Here, we present an unusual case of aortico-left ventricular tunnel associated with Takayasu's arteritis in a 44-year-old man.

Left aorto-ventricular tunnel: A differential diagnosis to aortic regurgitation.

Left aorto-ventricular tunnel is a rare congenital heart disorder. A then 19-year-old young lady was referred to our clinic for chronic rheumatic heart disease with aortic regurgitation. However, because of an almost continuous murmur, and an unusual transthoracic echo, she underwent various investigations, finally revealing a rare type 2 left aorto-ventricular tunnel. She had been variously diagnosed as chronic rheumatic heart disease with aortic regurgitation, bicuspid aortic valve with aortic regurgitation, and then possibly coronary arterial (cameral) fistula (based on 2D transthoracic echo) and finally left aorto-ventricular tunnel. This is the first case described in South East Asia who is asymptomatic, apart from a murmur, and her long journey to this final diagnosis illustrates the need to "chase shadows" on echoes, to come to a proper conclusion for the patient.

A newborn with aortico-left ventricular tunnel mimicking sinus of valsalva aneurysm.

Aortico-left ventricular tunnel (ALVT) is a rare congenital cardiac anomaly and constitutes less than 0.1% of all congenital cardiac defects (1). ALVT is described as an abnormal connection between the ascending aorta and the left ventricle which originates commonly above the right sinus of valsalva. Most patients are diagnosed with an ALVT during early infancy (2). Although transthoracic echocardiography (TTEAQ5) is more effective in diagnosis of ALVT, misdiagnosis rate was 17.1% (3). Sinus of valsalva aneurysm (SVA) is frequently confused with ALVT (3). We report a term female newborn with SVA in echocardiographic examination, but in surgery, she was diagnosed with ALVT.

Cierre de túnel aorto-ventricular derecho mediante un dispositivo percutáneo / Closure of the right aorto-ventricular tunnel through a percutaneous device

Resumen El túnel aorto-ventricular derecho es una anomalía congénita que consiste en un canal que conecta la aorta ascendente a la cavidad del ventrículo derecho. El diagnóstico se corrobora, en la mayoría de los casos, por ecocardiografía. El cierre quirúrgico se considera el tratamiento de elección y solo se han reportado dos casos previos en los que se ha logrado el cierre mediante intervencionismo. Se presenta el caso de una lactante con diagnóstico de túnel aorto-ventricular derecho, tratada por intervención percutánea con dispositivo de cierre percutáneo, que tuvo buena evolución clínica. La paciente, además, presentó un ductus permeable, el cual fue tratado en una segunda oportunidad por intervencionismo. La factibilidad de la técnica de cierre por procedimiento percutáneo y su baja tasa de complicaciones en comparación con el cierre por cirugía abierta pueden hacer de este el procedimiento de elección en la mayoría de los casos para esta infrecuente condición.

Abstract The aorto-right ventricular tunnel is a congenital anomaly that consists of a channel that connects the ascending aorta to the right ventricular cavity. The diagnosis is usually made by transthoracic echocardiography. Surgical closure is usually considered the treatment of choice and only two previous cases have been reported in which closure has been achieved by percutaneous intervention. We report a case of an infant with a diagnosis of aorto-right ventricular tunnel treated using a percutaneous device with good clinical evolution. The patient had also a patent ductus which was later treated by interventionism. The feasibility of the technique and its low rate of complications compared to the closure by open surgery can make it the procedure of choice in most cases for this rare condition.

Mid-term outcome of surgical treatment in patients with aorto-left ventricular tunnel.

OBJECTIVES: The purpose of this study was to review the outcomes of surgical treatment in patients with aorto-left ventricular tunnel and to investigate what kind of patient cohort is more likely to have adverse events. METHODS: Twenty-one patients with a median age of 6.58 [interquartile range (IQR) 4.17-24.50] years who received surgical treatment of aorto-left ventricular tunnel from March 2002 to December 2019 were reviewed. The median follow-up time was 64.50 (IQR 25.15-120.50) months. Clinical characteristics, surgical methods and follow-up outcomes were summarized in separate groups of patients with or without preoperative aortic valve (AoV) issues. Composite adverse events were defined as death or requirement of reoperation. Time-related analysis of freedom from death and requirement of reoperation was performed with the Kaplan-Meier method. RESULTS: The average tunnel size was 8.68 (standard deviation: 3.62) mm. The most common and the most important associated lesions were AoV lesions. Tunnels in 20 patients were closed with direct sutures or a patch. For 1 patient with an irreparable AoV, the tunnel was cut open simultaneously with aortic valve replacement and aortic root plasty. In the group of patients with preoperative AoV issues, 4 patients received aortic valve replacement with mechanical prosthetic valves and 6 patients received aortic valvuloplasty repair. The follow-up outcomes differed significantly between groups (the incidence of death was 15.38% and the incidence of requiring a reoperation was 46.15% in patients with preoperative AoV issues). In the group without preoperative AoV issues, there were no deaths and no reoperations (Fisher's exact test; P = 0.018). The probability of freedom from death and of the requirement for reoperation between the 2 groups was not significantly different (log-rank, P = 0.09). Overall, the estimated probability of freedom from death and requirement of reoperation was 77.30% (standard error: 10.20%) [95% confidence interval (CI): 49.53-91.00] at 5 years, 67.64% (standard error: 12.70%) (95% CI: 36.71-85.84) at 10 years. CONCLUSIONS: Patients with aorto-left ventricular tunnel with preoperative AoV issues are more prone to die or to require a reoperation. In contrast, patients without preoperative AoV issues can be free from death or reoperation for a longer period of time. Patients with preoperative AoV issues need much stricter postoperative long-term echocardiographic follow-up.

A case of a large type IV aortico-left ventricular tunnel successfully repaired at 1 day after birth.

A rare case of a patient with a large type IV aortico-left ventricular tunnel who underwent successful repair at 1 day after birth was described. To the best of our knowledge, no such cases that aortic opening was over 10 mm have been reported in the English literature. Our case demonstrated no leak or significant aortic regurgitation by a two-patch repair.

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.