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

Rad, Elaheh Malakan; Hijazi, Ziyad M.; Pouraliakbar, Hamidreza; Mirzaaghayan, Mohammad Reza; Zamani, Hossein

doi:10.1007/s00246-021-02671-5

Cited by 6 publications

(16 citation statements)

References 130 publications

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“…for separate lumen separate wall passage from the aorta, which are the aorto-atrial tunnels. 12 Therefore, further presurgical confirmatory imaging prior to surgery is mandatory. Invasive cardiac catheterization for angiocardiography was used in older case reports whenever diagnosis remains in doubt or for allocation of coronaries.…”

Section: Discussionmentioning

confidence: 99%

“…proposed a novel classification for aorto‐cardiac connectors into type 1, which is a hole‐like connector for rupture sinus of Valsalva aneurysm, type 2 as an anatomic passage for coronary cameral fistula, and type 3 for extranatomic passages. Type 3 is subclassified into A for separate lumen shared wall passage with the aorta, which are the aorto‐ventricular tunnels and B for separate lumen separate wall passage from the aorta, which are the aorto‐atrial tunnels 12 …”

Section: Discussionmentioning

confidence: 99%

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

Rakha,

Batouty,

Abdelrahman

et al. 2024

Echocardiography

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BackgroundAorto‐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 presentationA 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.ConclusionsMultimodality imaging can accurately assess cardiac function and demonstrate the anatomy of ALVT noninvasively to plan for successful intervention.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Rakha,

Batouty,

Abdelrahman

et al. 2024

Echocardiography

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“…A recent major attempt extracted all previous publications spanning 41 years across the world from 1980 to 2021 and enrolled 59 patients with this rare anomaly. [ 9 ] Conventional surgery closed either the aortic or the atrial end or both on cardiopulmonary bypass, though ligation of the tunnel on a beating heart could also arrest the shunt. [ 3 10 11 ] Transcatheter management involved antegrade or retrograde closure using coils, vascular plugs, or off-label use of duct occluders.…”

Section: Introductionmentioning

confidence: 99%

Aortico right atrial tunnel – Clinical presentation, transcatheter management, and follow-up from a large cohort of patients

Sivakumar,

Singhi,

Sankarakuttalam

et al. 2024

Annals of Pediatric Cardiology

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Background: Aortico right atrial tunnel (ARAT) is a rare extracardiac communication between the aorta and the right atrium with two anatomical types. A recent global review identified 59 patients. Methods: Patients with ARAT from two centers were analyzed for their demographics, symptoms, morphology, management, and follow-up thromboprophylaxis. Results: Among 21 patients including 8 males with a median age of 3 years (18 days–72 years) diagnosed as ARAT, 12 (57%) had posterior tunnels and 9 had anterior tunnels. Four patients had multiple exits. Eighteen tunnels were closed after arteriovenous circuit formation. Six patients (29%) weighing <10 kg presented early with heart failure. Transcatheter closure normalized the hemodynamics including in one infant after failed surgery. Two elderly patients (10%) above 60 years presented with angina and atrial fibrillation. The rest were asymptomatic. Occluders were positioned in the narrow proximal aortic end of the tunnel in all except two patients, where the distal atrial end was closed. All procedures were successful without complications. There was one late death after 1 year from subarachnoid hemorrhage. At a median follow-up of 96 months, all survivors were asymptomatic. Thromboprophylaxis with dual antiplatelets for 1–2 years followed earlier was recently changed to aspirin with Coumadin. Complete remodeling occurred when the proximal aortic end was closed, but partial persistence of the track was noted after distal closure. Conclusions: This largest cohort of ARAT showed the safety and efficacy of transcatheter closure even in neonates. The narrow proximal aortic end should be the target for closure rather than the distal atrial end to achieve complete remodeling.

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“…Aorto-left ventricular tunnel (ALVT) is an extracardiac paravalvular channel that connects the ascending aorta above the sinotubular junction with the cavity of the left ventricle [1,2]. ALVT is one of the rarest prenatally diagnosed cardiac defects and is part of an entity called abnormal congenital aorto-cardiac communications, a heterogeneous constellation of anomalies resulting in an abnormal connection between the aorta and other cardiac chambers or structures, namely rupture of congenital sinus of Valsalva aneurysm, aorto-left ventricular and less commonly right ventricular tunnels, coronary cameral fistulas, and aorto-atrial communications [3].…”

Section: Introductionmentioning

confidence: 99%

“…(3 mm at 18 weeks), antegrade flow over AV, to-and-fro flow in AoA, AA aneurysm, CHF, PE, HF Autopsy: AV dysplasia with stenosis TOP at 20.anterior (2.5 mm at 18 weeks), antegrade flow over AV, to-andfro flow in AoA, AA aneurysm, CHF, PE, pleural effusion Autopsy: AV dysplasia with stenosis, non-compacted LV myocardium TOP at 19.ALVT right anterior (3.7 mm at 21 weeks) antegrade flow over AV, to-and-fro flow in AoA, AA aneurysm, CHF, PE TOP at 21.5 weeks A V S A L V T l e f t a n t e r i o r ( 4 m m a t 3 0 w e e k s ) , antegrade flow over AV, to-and-fro flow in AoA, LOT aneurysm, AA aneurysm Autopsy: hypoplastic RCA, chronic ischemic (6 mm at 36 weeks), antegrade flow over AV, to-and-fro flow in AoA, AA aneurysm, CHF, PE, HF Dysplastic MV, non-compacted LV myocardium, severe CHF, pulmonary edema(6 mm at 30 weeks), no flow over AV, to-and-fro flow in AoA, LOT aneurysm, AA aneurysm Severe AS, non-compacted LV myocardium, (2 mm at 30 weeks), severe PS, antegrade flow over AV, antegrade flow in AoA, LOT aneurysm Origin of RCA in ALVT, normal LCA origin al. Aorto-Left Ventricular Tunnel… Ultraschall in Med 2023; 44: e184-e190 | © 2022.…”

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confidence: 99%

Aorto-Left Ventricular Tunnel – Prenatal Diagnosis and Outcome

et al. 2022

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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.

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

Cited by 6 publications

References 130 publications

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

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

Aortico right atrial tunnel – Clinical presentation, transcatheter management, and follow-up from a large cohort of patients

Aorto-Left Ventricular Tunnel – Prenatal Diagnosis and Outcome

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