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Ann Thorac Surg 2000;69:S164-S169
© 2000 The Society of Thoracic Surgeons
a Pediatric Cardiovascular Surgery, Children’s Hospital, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania, USA
Address reprint requests to Dr Myers, Pediatric Cardiovascular Surgery, Children’s Hospital at Milton S. Hershey Medical Center, PO Box 850, MC H085, Hershey, PA 17033
e-mail: jlmyers{at}psghs.edu
Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.
Abstract
The extant nomenclature for aortico-left ventricular tunnel is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The Hovaguimian classification appears most useful to surgeons: type 1, a simple tunnel with a slit-like opening at the aortic end, no aortic valve distortion; type II, a large extracardiac aortic wall aneurysm of the tunnel with an oval opening at the aortic end, with or without ventricular distortion; type III, intracardiac aneurysm of the septal portion of the tunnel, with or without right ventricular outflow tract obstruction; and type IV, a combination of type II and III. A comprehensive database set is presented, which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
I. Background
Aortico-left ventricular tunnel (ALVT) was first described in 1963 by Levy as an abnormal paravalvular communication between the aorta and left ventricle [1]. This anomaly is rare. The true incidence is unknown. Okoroma reported two cases among 1,754 patients with congenital heart disease, documented by cardiac catheterization, at operation, or at postmortem examination between 1964 and 1974 [2].
Bove and Schwartz have described their patients as having the aortic opening lateral or distal to the thick sinotubular ridge [3]. Hovaguimian and associates reviewed the literature and found that the aortic opening was above the right coronary artery ostium in 40% of patients, below the right coronary artery in 25% of patients, and at the level of the right coronary artery in 16% of patients [4].
Most often the aortic orifice of the aortico-left ventricular tunnel is anterior and just downstream from the sinotubular ridge above the right sinus of valsalva and right coronary artery [1–6]. This bulge can be seen externally. The tunnel passes downward beside the aortic valve and through the infundibular septum before entering the left ventricle. As it passes through the infundibular septum it can cause anterior displacement of the septum which can produce important subpulmonary stenosis [4]. The tunnel can communicate with the right ventricle, but this is very rare [7]. Most feel that when communication with the right ventricle is present, then the defect is really a ruptured sinus of valsalva aneurysm. Hovaguimian and colleagues in 1988 reviewed the literature and published a new classification [4]. Four types were described: type I, a simple tunnel with slitlike opening at the aortic end, no aortic valve distortion; type II, a large extracardiac aortic wall aneurysm of the tunnel with an oval opening at the aortic end, with or without valvular distortion; type III, intracardiac aneurysm of the septal portion of the tunnel, with or without right ventricular outflow tract obstruction; and type IV, a combination of types II and III (Fig 1).
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Patients with congenital aortico-left ventricular tunnel usually have severe aortic regurgitation. They present with severe congestive heart failure, cardiomegaly, and signs and symptoms of severe aortic regurgitation early in life [1–6, 8–10]. Diagnosis can now be usually made by echocardiography, and cardiac catheterization may not be necessary unless other associated cardiac defects are detected that require further investigation [6, 8–12].
Treatment
Medical treatment alone had almost always resulted in eventual death from severe heart failure, unless the defect was small [2, 4, 5]. Medical treatment, other than preoperative stabilization, is therefore not warranted. Operation has been the only successful approach to treatment of this anomaly. Different surgical techniques have been employed with the main objective to obliterate the abnormal communication and avoidance of any injury or distortion of the aortic valve [1, 2, 4, 5, 6, 8–11, 13, 14]. The surgical techniques described are direct suture closure of the aortic end of the tunnel, patch closure of the aortic, and a closure of both the aortic and the ventricular end of the tunnel. When surgical repair was delayed until an older age, distortion of the aortic valve frequently occurred, often necessitating replacement of the valve [5, 15, 16]. On the other hand, early repair has been recommended, and has been very successful with a low incidence of aortic valve problems [2, 4, 5, 10, 13]. Further long-term reports will be necessary in order to know the ultimate fate of these left ventricular outflow tracts.
II. Analysis: a unified aortico-left ventricular tunnel nomenclature system
Aortico-left ventricular tunnel hierarchy level 1
Aortico-left ventricular tunnel hierarchy level 1 definitions
Aortico-left ventricular tunnel hierarchy level 2
Aortico-left ventricular tunnel hierarchy level 2 definitions
Aortico-left ventricular tunnel hierarchy level 3
Aortico-left ventricular tunnel hierarchy level 3 definitions
III. Nomenclature for treatment options
Aortico-left ventricular tunnel treatment hierarchy level 1
Aortico-left ventricular tunnel treatment hierarchy level 1 definitions
Aortico-left ventricular tunnel treatment hierarchy level 2
Aortico-left ventricular tunnel treatment hierarchy level 3
IV. Outcome tables
Aortico-left ventricular tunnel surgery type (by year)
This table will show the number and percentage of each major aortico-left ventricular tunnel type (according to aortico-left ventricular tunnel hierarchy level 2) for each year. (All tables below will break down the data for each given year of data collection, and will also provide the total date of the cumulative experience).
Aortico-left ventricular tunnel: age [years] at operation for each aortico-left ventricular tunnel type
This table will show the distribution of age at operation for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: gender distribution for each aortico-left ventricular tunnel type (by year)
This table will show the gender for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: features of repair—cardiopulmonary bypass (by year)
This table will show the number of percentage of each major aorticoventricular tunnel type treated with cardiopulmonary bypass for each year.
Aortico-left ventricular tunnel: features of repair—aortic cross-clamp
This table will show the number and percentage of each major aortico-left ventricular tunnel type treated with aortic cross-clamping for each year.
Aortico-left ventricular tunnel: features of repair—induced fibrillation
This table will show the number and percentage of each major aortico-left ventricular tunnel type treated with induced fibrillation for each year.
Aortico-ventricular tunnel: features of repair—percent of patients having deep hypothermia and circulatory arrest
This table will show the number and percentages of each major aorticoventricular type treated with circulatory arrest for each year.
Aortico-left ventricular tunnel: features of repair—percent of patients less than 6 months having deep hypothermia and circulatory arrest
For patients less than 6 months, this table will show the number and percentage of each major aortico-left ventricular tunnel type treated with circulatory arrest for each year.
Aortico-left ventricular tunnel: features of repair—percent of patients greater than or equal to 6 months having deep hypothermia and circulatory arrest
For patients greater than or equal to 6 months, this table will show the number and percentage of each major aortico-left ventricular tunnel type treated with circulatory arrest for each year.
Aortico-ventricular tunnel: features of repair—myocardial preservation [cardioplegia type]
For patients treated with cross-clamping, this table will show the number and percentage of each major aortico-left ventricular tunnel type treated with various cardioplegia types, including blood, crystalloid, substrate enriched, and others.
Aortico-ventricular tunnel: features of repair—closure technique
This table will show the number and percentage of primary (suture) and patch closure for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: complication incidence (including operative death)
This table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system, including cardiac, pulmonary, renal, infectious, and neurologic) for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: complication incidence (including operative death) patients less than 1 year of age
For patients less than 1 year, this table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system, including cardiac, pulmonary, renal, infectious, and neurologic) for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: complication incidence (including operative death) patients greater than 1 year of age
For patients greater than or equal to 1 year, this table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system, including cardiac, pulmonary, renal, infectious, and neurologic) for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: preoperative length of ventilation (hours)
This table will show the preoperative length of ventilation for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: postoperative length of ventilation (hours)
This table will show the postoperative length of ventilation for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: total length of ventilation (hours)
This table will show the total length of ventilation for each major aorticoventricular type for each year.
Aortico-left ventricular tunnel: preoperative length of stay (days)
This table will show the preoperative length of stay for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: same day operation
This table will show the number and percentage of day of operation admissions for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: postoperative length of stay (days)
This table will show the postoperative length of stay for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: total length of stay (days)
This table will show the total length of stay for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: preoperative length of stay (days) by patient age
This table will show the preoperative length of stay for each major aortico-left ventricular tunnel type for each year, comparing patients less than 1 year to those greater than or equal to 1 year.
Aortico-left ventricular tunnel: same day operation by patient age
This table will show the number and percentage of day of operation admissions for each major aortico-left ventricular tunnel type for each year, comparing patients less than 1 year to those greater than or equal to 1 year.
Aortico-left ventricular tunnel: postoperative length of stay (days) by patient age
This table will show the postoperative length of stay for each major aortico-left ventricular tunnel type for each year, comparing patients less than 1 year to those greater than or equal to 1 year.
Aortico-left ventricular tunnel: total length of stay (days) by patient age
This table will show the total length of stay for each major aortico-left ventricular tunnel type for each year, comparing patients less than 1 year to those greater than or equal to 1 year.
Kaplan-Meier curves
Kaplan-Meier survival curves should be generated for each major aortico-left ventricular tunnel type for each year, comparing the total aortico-left ventricular tunnel cohort to patients less than 1 year to those greater than or equal to 1 year.
Aortico-left ventricular tunnel: complication incidence (including operative death) versus preoperative ventilation
This table will compare the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system, including cardiac, pulmonary, renal, infectious, and neurologic) in patients treated with and without preoperative ventilation, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: complication incidence (including operative death) versus deep hypothermia and circulatory arrest
This table will compare the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system including cardiac, pulmonary, renal, infectious, and neurologic) in patients treated with and without circulatory arrest, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: complication incidence (including operative death) versus myocardial preservation (cardioplegia type)
This table will compare the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system including cardiac, pulmonary, renal, infectious, and neurologic), in patients treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: complication incidence (including operative death) versus closure technique
This table will compare the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system including cardiac, pulmonary, renal, infectious, and neurologic), in patients treated with primary (suture), patch, and device closure, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: postoperative length of stay (days) versus preoperative ventilation
This table will compare the postoperative length of stay, in patients treated with and without preoperative ventilation, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: postoperative length of stay (days) versus deep hypothermia and circulatory arrest
This table will compare the postoperative length of stay, in patients treated with and without circulatory arrest, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: postoperative length of stay (days) versus myocardial preservation (cardioplegia type)
This table will compare the postoperative length of stay, in patients treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: postoperative length of stay (days) versus closure technique
This table will compare the postoperative length of stay, in patients treated with primary (suture), and patch closure, for each major aortico-left ventricular tunnel type for each year.
Aortico-left ventricular tunnel: freedom from reoperation
This table will show the freedom from reoperation for each major aortico-left ventricular type for each year.
References
This article has been cited by other articles:
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  L. A Bockeria, V. P Podzolkov, O. A Makhachev, and I. E Bondarenko Congenital aortico-left ventricular tunnel: anatomic variations and surgical experience Interactive CardioVascular and Thoracic Surgery, June 1, 2008; 7(3): 511 - 512. [Full Text] [PDF]
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