International Journal of Cardiology 70 (1999) 201–205
Aortico-right ventricular tunnel Sachin Talwar, Ujjwal Kumar Choudhary, Shyam Sunder Kothari, Balram Airan* Cardiothoracic Sciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India Received 19 January 1999; accepted 8 March 1999
Abstract A 2-year-old child with aortico-right ventricular tunnel is reported for its rarity. The right coronary artery originated from the distal end of the tunnel. The frequent occurrence of coronary artery origin abnormality with this anomaly is highlighted. 1999 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Aortico-right ventricular tunnel
1. Introduction Aorticoventricular (AV) tunnel is a rare congenital cardiac malformation, which presents as aortic runoff in infancy. Although more than 50 cases of aortico-left ventricular tunnel have been reported [1], only seven cases of aortico-right ventricular tunnel (ARVT) have been reported [2–8]. We report another case of ARVT and briefly review the literature.
2. Case report This 2-year-old male child was born after normal full term pregnancy. Birth weight was 3 kg. The child was apparently asymptomatic till 6 months of age. At 6 months of age, he developed cough, fever and tachypnoea suggesting congestive heart failure for which he was administered antibiotics, digoxin and diuretics. Subsequently, he continued to develop *Corresponding author. Tel.: 191-11-686-4851 / 656-1123; fax: 19111-686-2663. E-mail address:
[email protected] (B. Airan)
recurrent cough and fever and was referred to us at 2 years of age for evaluation. Heart rate of 140 per min, blood pressure of 80 / 30 mmHg, bilaterally symmetrical high volume peripheral pulses with no radiofemoral delay and hepatomegaly 2 cm below the right costal margin were noted. There was a prominent right ventricular impulse with systolic and diastolic thrills at the left lower sternal border and a loud ‘to and fro’ systolic and diastolic murmur was heard along the left sternal border. Plain chest roentgenography revealed cardiac enlargement with increased pulmonary vascular markings. Electrocardiogram showed right atrial and right ventricular enlargement. Two-dimensional echocardiography (2D echo) revealed a communication between the aorta and the right ventricle; a ruptured sinus of valsalva aneurysm was suspected. The right coronary artery was not seen. The patient had a detailed haemodynamic evaluation (Table 1) and angiocardiography. Angiography showed a well contracting enlarged left ventricle and filling of the right ventricle from the aortic root via a bulge above the region of the right coronary sinus. However, due to the dilution of the
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Table 1 Values obtained during pre-operative cardiac catheterization
Superior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary capillary Wedge pressure Left ventricle Aorta Femoral artery Pulmonary flow (l / mt) Systemic flow (l / mt) Pulmonary systemic flow ratio Pulmonary vascular resistance (Wood units)
Oxygen saturation (%)
Pressure (mmHg)
74.2 69.8 83.6 92.9
a8–12 V4–8 mean 5 35-39 / 12 32 / 12-14 mean 19
96.8 97.7 97.7 21.16 5.67 4.61 0.22
a13–19 V12–16 mean 14 110 / 22 100 / 50 mean 66
Fig. 1. Aortic root angiogram (right anterior oblique view) showing communication (C) between the aorta (AO) and the faintly opacified right ventricle (RV). The right coronary artery (arrow) is seen to originate from the right ventricular end of the communication.
S. Talwar et al. / International Journal of Cardiology 70 (1999) 201 – 205
contrast and rapid run-off, details could not be made out. The right coronary artery was seen to arise from the right ventricular end of this bulge (Fig. 1). There was no ventricular septal defect or patent ductus arteriosus. A ruptured sinus of valsalva aneurysm or an ARVT was suspected and the patient was taken up for surgery. At operation, the heart was enlarged with the enlargement of the right ventricle and distension of the pulmonary artery. There was an external outpouching of the aorta just above the region of the right coronary sinus. This was continuous with a membranous structure beneath the aorta, which continued into a bulge into the right ventricular outflow tract. On palpation, a prominent systolic and diastolic thrill was felt over this structure and the right ventricular outflow tract. Standard moderate hypothermic cardiopulmonary bypass was established, aorta was cross-clamped and St. Thomas cardioplegia solution was administered into the aortic root while the bulge was occluded with the finger. A standard oblique aortotomy was made. All aortic sinuses were normal; the aortic annulus was normally formed and the aortic valve cusps had a normal structure and configuration. An oval opening |1.0 cm in diameter was present about 0.5 cm above the upper margin of the right sinus of valsalva. This communicated with a tunnel |1.5 cm long that entered the infundibulum of the right ventricle. Lining of the tunnel appeared similar to that of the aorta. The normal sized right coronary ostium was located at the anterior end of the orifice of the tunnel towards the right ventricle (Fig. 2). The left coronary ostium was located normally. Both the coronary arteries were normal in distribution. Passage of a probe through the tunnel into the right ventricular infundibulum confirmed the diagnosis of an aortico-right ventricular tunnel. The defect was closed with a Gore-Tex (W.L. Gore, Flagstaff, AZ) patch with 5-0 polypropylene at the level of its entry into the right ventricle taking care not to obstruct the right coronary ostium. Postoperatively, the child was haemodynamically stable and made an uneventful recovery and was extubated on the first postoperative day. However, 1 h after extubation he developed generalized convulsions and had to be put back on ventilatory support. Computerized axial tomographic scan of the head showed
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Fig. 2. Schematic diagram showing the tunnel (T) between aorta (AO) and right ventricle (RV). Note the origin of the right coronary artery (RCA) from near the right ventricular end of the tunnel. The left coronary artery ostium (LCA) is normally placed. PA, pulmonary artery; TV, tricuspid valve.
diffuse hypoxic encephalopathy. Despite stable haemodynamics, he developed cerebral edema and brain stem compression and died on the 14th postoperative day. Permission for autopsy was denied.
3. Discussion Communications between the aorta and any of the four cardiac chambers have been reported. However, aortico-right ventricular tunnel (ARVT) is extremely rare and only seven cases have been described in the English literature [2–8] (Table 2). The condition is more common in males and presents early in infancy with a to-and-fro murmur, bounding peripheral pulses, cardiomegaly. The majority of the patients have congestive heart failure and a precordial systolic and diastolic thrill. Haemodynamically, the anomaly produces an obligatory left to right shunt which is independent of the pulmonary vascular resistance [9], thus resulting in pressure and volume overload of the right ventricle
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Table 2 Summary of cases of ARVT in English literature Year
Author
Age
Sex
Associated anomaly
Coronary artery anatomy
Surgical procedure
Outcome
1973
Bharati [2]
1 day
Male
–
–
1974
Saylam [3]
10 years
Male
–
RCA from ARVT Absence of LCA ostium
Autopsy diagnosis Survived
1989
Jureidini [4]
1 day
Female
Critical PS, PDA
Normal
1992
Kleikamp [5]
5 days
Male
LCA from ARVT
1992
Westaby [6]
3 months
Male
Rudimentary valve of ARVT PFO, PDA –
1993
Rosengart [7]
3 months
Female
PS
Absent RCA ostium
1998
VanSon [8]
15 months
Male
–
Normal
Normal
Closure of aortic and ventricular ends of tunnel Pericardial patch closure of aortic opening of tunnel, PDA ligation pulmonary valvotomy Direct closure of tunnel and PFO, PDA ligation Goretex patch closure of aortic end of tunnel reinforced with pericardium Goretex patch closure of aortic and ventricular ends of tunnel, pulmonary valvotomy Direct closure of aortic and ventricular openings of ARVT
Died
Died
Survived
Died
Survived
PS, pulmonary stenosis; LCA, left coronary artery; RCA, right coronary artery; PDA, patent ductus arteriosus; PFO, patent foramen ovale.
and rapid clinical deterioration in the majority of the cases. However, as more cases of this anomaly are described, patients with less severe clinical manifestations may also be encountered. These clinical and pathological features resemble those of ruptured sinus of valsalva aneurysm and coronary arterio venous fistula. AV tunnel presents in the first few days of life whereas ruptured sinus of valsalva is more common in the second to fourth decade of life. Consistency and thickness of ARVT is similar to that of the aortic wall and an externally visible bulge is seen on the external aspect of the aorta. It then passes either into the left ventricle or rarely the right ventricle. Coronary artery anomalies are common and include right coronary artery from ARVT, absence of left coronary artery ostium, left coronary artery from ARVT and absence of right coronary artery ostium [2,3,5,7]. On the other hand, ruptured sinus of valsalva is thin walled, narrow and
runs an entirely intracardiac course to the cardiac chamber. It may be almost impossible to differentiate between the two conditions on clinical examination, echocardiography and angiography as in the present case. It is possible to identify ARVT pre-operatively if on angiography passage of contrast medium is observed through an orifice above the aortic cusps whereas ruptured sinus of valsalva is at the level of the cusps. It is also difficult to differentiate ARVT from a very proximal right coronary fistula because clinical features are the same and on angiography the contrast medium is rapidly disseminated from the aorta [6]. Differentiation with coronary artery fistula on 2D echo is based upon visualization of coronary arteries. In coronary artery fistula the involved coronary artery is very dilated while in ARVT the coronary arteries are normal. The pathogenesis of ARVT is unclear. It may be
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due to persistence of embryonic ventricular sinusoids that open into the aortic sinuses [2,4,10]. However some feel that it is either a coronary anomaly or a malformation of the distal bulbus cordis [11]. In fact, coronary artery anomalies have been observed in four out of the seven reported cases in the literature [2,3,5,7]. In our case also, the right coronary artery originated from the right ventricular end of the tunnel. This leads us to believe that ARVT may be a form of coronary artery anomaly. ARVT leads to rapid deterioration of cardiac function and death as has been seen in four out of the seven described cases [2,4,5,7]. Hence, surgical intervention is required for all cases at the earliest to reduce the left to right shunt and volume overload of the right ventricle and also to prevent development of irreversible pulmonary vascular disease. Particular attention should be directed towards recognition of a coronary artery anomaly as aberrant coronary artery origin may prevent successful surgical correction. The surgical options have included direct closure of the aortic end of the tunnel or closure of both the aortic and ventricular ends. In case there are major coronary anomalies, closure is performed at the right ventricular end of the tunnel [5]. Patch closure of the aortic end is indicated in the presence of a short tunnel and a large aortic orifice of the tunnel [6,7]. In patients with aberrant origin of the coronary artery from the tunnel, the closure of the ventricular end of
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the tunnel should be performed through an aortotomy so that the coronary artery origin is visualized and protected.
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