Ann Thorac Surg 2008;86:1682-1684. doi:10.1016/j.athoracsur.2008.04.096
© 2008 The Society of Thoracic Surgeons
Case Reports
Hybrid Transcatheter-Surgical Strategy in Arterial Tortuosity Syndrome
Giuseppe Santoro, MDa,*,
Giuseppe Caianiello, MDc,
Giovanni Rossi, MDa,
Gabriella Farina, MDc,
Maria Giovanna Russo, MDb,
Raffaele Calabrò, MDa
a Department of Cardiology, A. O. "Monaldi" 2nd University of Naples, Naples, Italy
b Department of Radiology, A. O. "Monaldi" 2nd University of Naples, Naples, Italy
c Department of Pediatric Cardiac Surgery, A. O. "Monaldi" 2nd University of Naples, Naples, Italy
Accepted for publication April 25, 2008.
* Address correspondence to Dr Santoro, Via Vito Lembo, 14-Salerno, 84131, Italy (Email: santoropino{at}tin.it).
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Abstract
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Arterial tortuosity syndrome is a rare connective tissue disorder characterized by elongation and tortuosity of large elastic arteries resulting in multiple vascular stenoses. This article reports on a novel hybrid transcatheter-surgical strategy to treat bilateral peripheral pulmonary artery stenoses due to this syndrome. Operation consisted of stent implantation by midline sternotomy and reductive reconstruction of the proximal pulmonary arteries. This approach resulted in right ventricular overload relief and sudden improvement of the patient's clinical condition.
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Introduction
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Arterial tortuosity syndrome is a rare autosomal recessive connective tissue disorder characterized by facial, skin, and joint abnormalities, as well as generalized elongation and tortuosity of the major elastic arteries [1, 2]. Pulmonary artery redundancy may lead to kinking and severe stenosis of the hilar pulmonary arteries [3–5] for which surgical or endovascular treatment is quite challenging. This article describes a novel hybrid transcatheter-surgical approach to this uncommon type of obstruction, consisting in stent implantation through a midline sternotomy followed by reductive reconstruction of the pulmonary arteries during cardiopulmonary bypass.
A 3-year-old child was referred to cardiac evaluation due to easy fatigability and cardiac murmur. He showed several mild phenotypic abnormalities (ie, down-slanting palpebral fissures, hypertelorism, joint hyperlaxity, and muscle hypotonia) and a soft cardiac systolic murmur widely irradiated over the chest. A chest roentgenogram showed mild cardiac enlargement without pulmonary vascular marking abnormalities. At electrocardiogram, right atrial dilatation and right ventricular hypertrophy signs were found. Echocardiography showed a severe right ventricular enlargement and pressure overload due to elongation of the main pulmonary branches and severe, bilateral peripheral pulmonary artery stenoses (peak-pressure gradient, 60 mm Hg with diastolic run-off). Also, the aortic arch was moderately elongated and tortuous, thereby resulting in mild aortic coarctation. This constellation of phenotypic and cardiovascular abnormalities caused us to diagnose the arterial tortuosity syndrome. Cardiac catheterization and a spiral computed tomographic scan showed that the pulmonary artery stenoses were caused by redundancy of the proximal pulmonary arteries, resulting in kinking and growth failure of their peri-hilar segments (Figs 1 and 2).
This anatomic arrangement was deemed unsuitable for surgical repair or simple stent angioplasty. Thus, after parental informed consent, a hybrid transcatheter-surgical approach to relieve the stenoses and shorten the redundant pulmonary arteries was attempted. Under general anaesthesia in the cardiac catheterization laboratory, the stenting procedure was performed through a midline sternotomy using pre-mounted stents introduced through a stab incision of the main pulmonary artery. The left pulmonary artery was dilated to 10 mm (Wavemax stent; Abbott Vascular, Beringen, Switzerland) and the stenoses of the upper and lower branches of the right pulmonary artery were dilated to 5 mm and 7 mm (Express Vascular stent; Boston Scientific, Galway, Ireland), respectively. The stents were chosen to be potentially further dilated in time with the growth of the patient. Then the child was taken to the operative room, where reductive pulmonary artery reconstruction during normothermic beating-heart cardiopulmonary bypass with bi-caval cannulation and pulmonary artery trunk cross clamping was performed. After the incision of the pulmonary artery trunk, a V-shaped segment of the inner circumference of the main pulmonary artery branches was removed and the resultant defect was closed using a continuous 6.0 Prolene suture (Ethicon, Somerville, NJ) (Fig 3). The postoperative course was uneventful and the pre-discharge cardiac catheterization showed an effective straightening of the pulmonary arteries without any pressure gradient across the stents (Fig 4), resulting in a significant decrease of the right-to-left ventricular pressure ratio (1.1 to 0.6). At 6 months from the procedure, the child is in functional New York Heart Association functional class I without any pharmacologic support.
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Fig 1. Moderate aortic arch tortuosity and pseudo-coarctation, as shown by spiral computed tomographic scan. (AAo = ascending aorta; MPA = main pulmonary artery; TA = transverse aortic arch.)
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Fig 2. (A) Pulmonary artery angiography showing a severe elongation and kinking of the main pulmonary arteries resulting in bending of their hilar segments (arrows). (B) Severe, discrete stenosis of the left pulmonary artery (black arrow), as imaged by spiral computed tomographic scan. (C) Multiple, long stenoses of the right pulmonary artery branches (white arrows), as imaged by selective intraoperative pulmonary angiography. (DA = descending aorta; LPA = left pulmonary artery; MPA = main pulmonary artery; RPA = right pulmonary artery.)
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Fig 3. Line drawing of the surgical procedure. (A) The incision of the pulmonary trunk and the main pulmonary arteries is followed by removal of a segment of their inner circumference (dashed area). (B) The pulmonary arteries after this reductive reconstruction.
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Fig 4. Pulmonary angiography after the hybrid transcatheter-surgical approach. (* = left pulmonary artery; ** = right pulmonary artery; MPA = main pulmonary artery.)
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Comment
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Arterial tortuosity syndrome is a rare connective tissue disorder with autosomal recessive inheritance, presumably due to loss-of-function mutations of genes located on chromosome 20q13 and encoding for facilitative glucose transporters [1, 6]. The resulting metabolic pathway changes lead to disruption of the vessel elastic network, and hence to weakness of the major elastic arteries that eventually result in their generalized elongation and tortuosity. Clinical evidence of this anomaly may derive from aneurysmal dilatation of the ascending aorta, aortic pseudo-coarctation or peripheral pulmonary artery stenoses [3–5]. In this case, the obstruction is due to redundancy of the proximal pulmonary arteries that leads to bending and growth failure of their peripheral segments. This anatomic feature precludes any effective treatment by either surgical enlargement or stent angioplasty. In fact, due to discrepancy between the elongated proximal pulmonary arteries and the mediastinal cavity, surgical repair or even extremely flexible stents could just move the site of the stenosis. Moreover, an incomplete relief of the peripheral pulmonary stenoses might hasten the progressive kinking of the proximal pulmonary arteries. Thus, in our opinion, the stent implantation should always be followed by reductive surgical reconstruction of the proximal pulmonary arteries to straighten the pulmonary pathway and effectively break up the previously mentioned vicious circle. We believe that this approach has never been reported in literature and should always be kept in mind in these complex vascular obstructions, mainly in low-weight patients. However, only a close follow-up will disclose us the long-term outcome of these vessels, as well as the need of further surgical or percutaneous interventions as the infant grows. Nevertheless, the use of stents potentially re-dilatable up to the adult pulmonary artery size, as was in this patient, should allow additional nonsurgical dilatations whenever the clinical conditions warranted them.
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References
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- Franceschini P, Guala A, Licata D, Di Cara G, Franceschini D. Arterial tortuosity sindrome Am J Med Genet 2000;91:141-143.[Medline]
- Zaidi SHE, Peltekova V, Meyer S, et al. A family exhibiting arterial tortuosity syndrome displays homozygosity for markers in the arterial tortuosity locus at chromosome 20q13 Clin Genet 2004;67:183-188.
- Hoop R, Steinmann B, Valsangiacomo Buechel ER. Cardiovascular findings in arterial tortuosity syndrome Eur Heart J 2006;27:2045.[Free Full Text]
- Bottio T, Bisleri G, Piccoli P, Muneretto C. Valve-sparing aortic root replacement in a patient with a rare connective tissue disorder: arterial tortuosity syndrome J Thorac Cardiovasc Surg 2007;133:252-253.[Free Full Text]
- Rivera IR, Gomes L, Moises VA, Silva CC, Andrade JL, Carvalho AC. Multiple arterial anomalies in the newborn infant. Echocardiographic and angiographic diagnosis. Arq Bras Cardiol 2000;75:141-144.
- Coucke PJ, Willaert A, Wessels MW, et al. Mutations in the facilitative glucose transported GLUT10 alter angiogenesis and cause arterial tortuosity sindrome Nat Genet 2006;38:452-457.[Medline]
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Abstract
Introduction
