Aortico-left Ventricular Tunnel Research Articles

A successful repair of prenatally diagnosed aortico-left ventricular tunnel

A successful repair of prenatally diagnosed aortico-left ventricular tunnel

The aorto-ventricular tunnels

LEVY AND COLLEAGUES, IN 1963, WHO ARE generally credited with the first description of “aortico-left ventricular tunnel”.1 Examples of the malformation, nonetheless, were illustrated initially by Burchell and Edwards in 1957,2 and by Edwards in 1961.3 The subsequent documentation of more than 130 cases has now elucidated many features of the so-called “tunnels,” including their clinical presentation and surgical management. While most of the abnormal channels extend between the aorta and the left ventricle,4–79 it is now also recognized that some, alternatively, enter the right ventricle.80–91 The anatomic arrangement underscoring the malformations has been clarified by recent morphological studies,92,93 while diagnosis during fetal life has established beyond any doubt that the lesions are congenital.42,43 Although rare, the aorto-ventricular tunnel is the foremost cause during infancy of regurgitant flow of blood from the aorta to one or the other of the ventricles. In this review, we will describe and illustrate the structure of the malformations, speculate upon their developmental basis, and discuss pertinent aspects of their diagnosis and treatment.

Successful two patch repair of the aortico-left ventricular tunnel

A 7-month-old boy with aortico-left ventricular tunnel arising from the right coronary sinus underwent surgical correction using the two patch technique. The diagnosis was confirmed by 2D and Doppler echocardiogram alone. Patch closure of both the aortic and ventricular ends of the tunnel is recommended in order to prevent postoperative aortic regurgitation and recurrence of the lesion.

Prenatal diagnosis of an aortico-left ventricular tunnel.

Aortico-left ventricular tunnel is a rare abnormal communication between the ascending aorta and the left ventricle. Early diagnosis and prompt surgical repair prevent congestive heart failure or consecutive valvular insufficiency. However, in utero diagnosis of this heart malformation has been rarely described and none of those children survived. We report on a case of aortico-left ventricular tunnel detected at 32 weeks' gestation with a favourable outcome after surgical repair at three months of age.

Congenital Heart Surgery Nomenclature and Database Project: aortico-left ventricular tunnel

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.

Repair of Aortico–Left Ventricular Tunnel Originating From the Left Aortic Sinus

We report on a case of an 11-year-old asymptomatic child with aortico–left ventricular tunnel arising from the left aortic sinus. Preoperative transesophageal echocardiography showed a dilated aortic root with mild aortic valve incompetence and demonstrated the course of the tunnel, which originated from the left coronary sinus entering the outlet portion of the left ventricular outflow tract. Patch closure of the aortic end of the tunnel eliminated left ventricular volume overload with immediate marked reduction of cardiomegaly. At 10-month follow-up the child is asymptomatic and receiving no oral medications. Control two-dimensional Doppler echocardiography shows trivial central aortic valve incompetence.

Aortico-Left Ventricular Tunnel: Late Reoperations

Aortico-left ventricular tunnel is a rare congenital anomaly that presents as aortic regurgitation in infancy or childhood. Information on late results of surgery, especially with reoperations for progressive aortic regurgitation and recurrences are limited. The case histories of two patients with reoperations following correction of aortico-left ventricular tunnel are reported.

Aortico-left ventricular tunnel: late reoperations

Aortico-left ventricular tunnel is a rare congenital anomaly that presents as aortic regurgitation in infancy or childhood. Information on late results of surgery, especially with reoperations for progressive aortic regurgitation and recurrences are limited. The case histories of two patients with reoperations following correction of aortico-left ventricular tunnel are reported.

Aortico-left ventricular tunnel in fetuses and infants

Aortico-left ventricular tunnel is a rare congenital abnormal communication between the aorta and the left ventricle presenting in early childhood as aortic regurgitation and cardiac failure. This condition has rarely been reported in fetuses. Operation is the only treatment, and postoperative aortic incompetence could be related to the age or the type of repair. We conducted a retrospective, two-institution review, from 1983 to 1995, of aortico-left ventricular tunnel diagnosed in utero and before 6 months of age. Three cases of aortico-left ventricular tunnel were diagnosed in utero by Doppler echocardiography between 22 and 24 weeks' gestation. Prenatal aortico-left ventricular tunnel was associated with severe left ventricular dysfunction, aortic valve anomalies, and fetal hydrops. One death occurred in utero and one immediately after birth, and in 1 case pregnancy was interrupted. In these 3 cases the diagnosis was confirmed by autopsy. Three neonates and 2 infants had the diagnosis of aortico-left ventricular tunnel made after birth and underwent successful surgical repair. At short and midterm follow-up all patients are alive and aortic valve regurgitation is absent or trivial. This series shows that aortico-left ventricular tunnel covers an anatomic spectrum of lesions. Cases diagnosed in utero by Doppler echocardiography are characterized by severe ventricular dysfunction, associated aortic valve lesions, and poor outcome. Postnatal cases represent the more favorable end of the spectrum, with no associated lesions, and can be repaired without mortality and with good functional results.

Aortico-left ventricular tunnel with aortic atresia in the newborn

Aortico-left ventricular tunnel with aortic atresia in the newborn

Repair of aortico-left ventricular tunnel associated with subpulmonary obstruction

Aortico-left ventricular tunnel is a rare congenital cardiac defect. Because the tunnel traverses the upper portion of the interventricular septum, the potential exists for an aneurysmal tunnel to obstruct the outflow of the right ventricle. We report our clinical experience with such a patient, and emphasize the importance of a two-patch technique to avoid its occurrence.

Aortico-left ventricular tunnel in a neonate

Aortico-left ventricular tunnel is a rare paravalvular communication between the aorta and the left ventricle resulting in severe aortic regurgitation. It originates most frequently just superior to the right sinus of Valsalva and enters the left ventricle just inferior to the right aortic valve cusp traversing the infundibular or perimembranous ventricular septum. We report our experience with this lesion presenting in a neonate on the first day of life and describe its clinical presentation, noninvasive diagnosis, and surgical management.

Aortico-left ventricular tunnel associated with critical aortic stenosis in the newborn

Aortico-left ventricular tunnel is a rare congenital malformation. A unique case of critical aortic stenosis in a neonate accompanying aortico-left ventricular tunnel is described. Successful surgical correction was performed without invasive studies following echocardiographic recognition of the defect.

Aortico-left ventricular tunnel: Long-term outcome after surgical repair

Over a 14 year period, four children (three male, one female) underwent surgical correction of an aortico-left ventricular tunnel. All presented in infancy (age range 5 days to 9 months). The presenting feature was a systolic and diastolic murmur in all, one of whom developed heart failure within 2 weeks of presentation. In the first two patients, the echocardiographic findings were inconclusive and the diagnosis was confirmed at cardiac catheterization (at 10 and 23 months of age, respectively); the other two were diagnosed echocardiographically by two-dimensional and Doppler color flow imaging.All four patients underwent surgery by patch closure of the aortic end of the tunnel (three patients) or direct suture closure (one patient) and there were no deaths. The mean age at operation was 11 months. During a mean follow-up period of 71 months (range 2 to 157), three patients have clinical and echocardiographic evidence of trivial aortic valve regurgitation, which was noted in the immediate postoperative period in one and at early (<6 months) follow-up study in the other two. All we symptomfree, are taking no medications and are growing and developing normally.Aortico-left ventricular tunnel can be accurately diagnosed by echocardiography. In patients presenting in infancy, echocardiography also provides the necessary morphologic information to enable surgical correction without angiography. Early operation is associated with an excellent outcome, whereas repair at a later age is associated with a high incidence of residual aortic regurgitation requiring further surgery.

Surgical treatment of aortico-left ventricular tunnel

Thirteen children (age range 6 h-11 years, median 4 months) operated upon for aortico-left ventricular tunnel since 1971 are reported. All patients had systolic and diastolic murmurs, 4 of 5 neonates were in severe congestive heart failure. Twelve children had left ventricular hypertrophy on ECG with an ischaemic pattern in the neonates. Chest X-ray revealed cardiomegaly with a broad upper mediastinal shadow from the enlarged aorta. Echocardiography (2DE) revealed a tubular communication between the aorta and the left ventricle bypassing the aortic valve and massive regurgitation through this tunnel demonstrated by Doppler sonography. In 8 patients, the diagnosis was confirmed by angiography. The tunnel arose from the right coronary sinus in 12 and from the left coronary sinus in 1 patient. The tunnel was closed at both ends in 8, at the aortic end only in 3 and at the ventricular end in the remaining 2 cases. One 6-day-old infant, operated upon in 1971 died postoperatively. There was 1 non-cardiac related late death due to a brain tumour. Echocardiographic analysis of left ventricular size and function was carried out in 9 children up to 39 months after surgery. Mild to moderate aortic incompetence was detected during the follow-up period in 2 of 12 patients. All surviving children are leading normal lives.

Surgical treatment of aortico-left ventricular tunnel

Thirteen children (age range 6 h-11 years, median 4 months) operated upon for aortico-left ventricular tunnel since 1971 are reported. All patients had systolic and diastolic murmurs, 4 of 5 neonates were in severe congestive heart failure. Twelve children had left ventricular hypertrophy on ECG with an ischaemic pattern in the neonates. Chest X-ray revealed cardiomegaly with a broad upper mediastinal shadow from the enlarged aorta. Echocardiography (2DE) revealed a tubular communication between the aorta and the left ventricle bypassing the aortic valve and massive regurgitation through this tunnel demonstrated by Doppler sonography. In 8 patients, the diagnosis was confirmed by angiography. The tunnel arose from the right coronary sinus in 12 and from the left coronary sinus in 1 patient. The tunnel was closed at both ends in 8, at the aortic end only in 3 and at the ventricular end in the remaining 2 cases. One 6-day-old infant, operated upon in 1971 died postoperatively. There was 1 non-cardiac related late death due to a brain tumour. Echocardiographic analysis of left ventricular size and function was carried out in 9 children up to 39 months after surgery. Mild to moderate aortic incompetence was detected during the follow-up period in 2 of 12 patients. All surviving children are leading normal lives.

Aortico-left ventricular tunnel: Successful diagnostic and surgical approach to the oldest patient in the literature

Aortico-left ventricular tunnel: Successful diagnostic and surgical approach to the oldest patient in the literature

Asymptomatic aortico-left ventricular tunnel in adulthood

Aortico-left ventricular (LV) tunnel is a rare congenital cardiac malformation. Fewer than 50 cases have been described 1–6 and only 3 in individuals over 20 years of age. 1,4,5

Correction of aortico-left ventricular tunnel during the first day of life

Two critically ill newborns with aortico-left ventricular tunnel and severe heart failure were operated on at six and 14 hours alter birth. The diagnosis was established clinically by the auscultatory finding of systolic and diastolic murmurs and by two-dimensional and Doppler echocardiography. In the first newborn, the left aortic sinus was connected with the left ventricle below the aortic valve by an aneurysmatically dilated tunnel. In the second patient, the tunnel connected the right aortic sinus and the left ventricle. The repair was performed under deep hypothermia, total hemodilution, and cardiopulmonary bypass. The tunnel was clused with two patches of Gore-Tex on the aortic and ventricular orifices. Both children are free from symptoms and are developing normally 10 and 8 months after repair.

Aortic atresia and aortico–left ventricular tunnel: Successful surgical management by Konno aortoventriculoplasty in a neonate

A newborn infant with aortic atresia and aortico-left ventricular tunnel was successfully treated by Konno aortoventriculoplasty. A 15 mm Ionescu-Shiley pericardial xenograft was inserted when the patient was 5 days old and it was replaced with a 19 mm St. Jude Medical prosthesis at 2 years of age.