Cardiology

Congenital Intervention - PDA, Coarctation, Pulmonary AVM, and Coronary Fistulae

General description of procedure, equipment, technique

Patent ductus arteriosus

A patent ductus arteriosus (PDA) accounts for 5% to 10% of all congenital heart disease and, if left unclosed, can lead to congestive heart failure, pulmonary hypertension, or infective arteritis. Currently, most PDAs can be safely and effectively closed percutaneously using coils or other devices, including the Amplatzer Ductal Occluder (ADO) (AGA Medical, Golden Valley, MN).

Amplatzer duct occluder use was first reported in 1998 and since then it has proven especially effective in closing moderate to large PDAs. The ADO has a nitinol frame that is packed with polyester fabric to enhance thrombosis. The elasticity of the nitinol facilitates device closure of a variety of ductal shapes and sizes. Following a large multicenter series, the device received Food and Drug Administration approval in 2003 for use in the United States. For the adult patient with patent ductus arteriosus, the ADO is frequently used for closure.

Coarctation of the aorta

Coarctation of the aorta is a congenital narrowing of the aorta, accounting for 5% to 8% of all congenital heart disease. The obstructive lesion occurs just distal to the origin of the left subclavian artery, where the fetal ductus arteriosus had previously inserted into the aorta.

The obstruction is usually a discrete, tubular narrowing, but common anatomic variations include long segment stenosis and transverse aortic arch hypoplasia proximal to the coarctation. Histologic abnormalities of the medial layer can be seen in the affected area. Between 50% and 85% of patients with coarctation also have a bicuspid aortic valve.

Although coarctation is most frequently diagnosed in the child presenting with hypertension, with diminished pulse volume in the lower extremities or with a heart murmur (either from the coarctation itself or from the associated bicuspid aortic valve), coarctation may go undetected well into adulthood.

Pulmonary arteriovenous malformation

Pulmonary arteriovenous malformations (AVMs) are abnormal connections between pulmonary arterial and venous systems resulting in a shunt to the left atrium that bypasses the small arterioles and the air containing spaces of the lung. Pulmonary AVMs occur most frequently as part of the Hereditary Hemorrhagic Telangiectasia syndrome (HHT), an autosomal dominant disorder, and may occur as isolated or multiple defects.

Isolated pulmonary AVMs may also be congenital in nature, or secondary to trauma or infection. Small, pulmonary AVMs distributed diffusely throughout the lung fields may be a product of pulmonary parenchymal disease, or the result of end-stage liver disease. In complex congenital heart disease patients, diffuse small pulmonary AVMs may occur with a long-standing Glenn shunt from the superior vena cava to pulmonary artery due to loss of hepatic venous return to the lungs. Also, approximately 3% to 5% of patients with migraine headaches with aura have been found to have a pulmonary AVM.

Coronary artery fistulae

Communications between the coronary arteries and the cardiac chambers are known as coronary-cameral fistulae; between the coronary arteries and low pressure veins they are known as coronary arteriovenous malformations.

These abnormalities are most often congenital but can also be acquired secondary to trauma or invasive procedures. Although coronary fistulae can arise from any of the coronary artery vessels, drainage is most often to either the right ventricle, right atrium, or the pulmonary arteries, and less frequently to the superior vena cava, coronary sinus, pulmonary veins or the left atrium.

Indications and patient selection

Patent ductus arteriosus

There are several clinical indications for intervention in adult patients with PDA, including a significant left-to-right shunt with left heart overload or a history of endarteritis at the PDA or adjacent structures. There are no data to suggest that closure of a small, nonvolume loading PDA, in an asymptomatic adult is necessary.

Coarctation of the aorta

Current indications for intervention in an adult patient include clinical symptoms, hypertension at rest, or severe hypertension with exertion usually a systolic right arm blood pressure above 220 mm Hg during exercise or recovery. Due to systemic hypertension in the upper body, these individuals are predisposed to premature coronary artery disease and cerebral berry aneurysm (3% to 5%).

Adults typically present with refractory hypertension, but may also complain of headaches, nosebleeds, cool extremities, fatigue, and leg weakness/claudication with exertion. They may also develop iatrogenic problems related to antihypertensive therapy (claudication or prerenal azotemia). Less common clinical manifestations include angina, exertional dyspnea, and heart failure from chronically increased left ventricular afterload. For the adult with unrepaired, isolated coarctation the average survival is approximately 35 years of age with a 25% survival beyond 50 years of age.

Pulmonary arteriovenous malformation

In the presence of clinical cyanosis, transcatheter closure is the treatment of choice for individual pulmonary AVM. The choice of closure device depends entirely on the size and location of the AVM to be closed. Multiple pulmonary AVMs, such as those seen with HHT or in end-stage liver disease, are difficult to treat medically. Closure of pulmonary AVMs to prevent recurrent paradoxical embolization and migraine headache remain unproven, similar to PFO closure.

Coronary artery fistulae

Small fistulae in asymptomatic patients should be followed clinically for signs of growth and increasing flow. Large, hemodynamically significant fistulae should be closed electively at the time of diagnosis. Various transcatheter occlusion techniques have been reported with excellent outcomes for fistulae with shorter, less tortuous courses.

Contraindications

Patent ductus arteriosus

Although there are not true contraindications for this procedure, there are patients with difficult access or total occlusion of the femoral vessels that the procedure may not be successful.

Coarctation of the aorta

Although there are no true contraindications, caution should be taken if the patient is older than 60 years, or if the lesion is atretic, severely stenotic, or resistant to balloon testing. In these settings, use of a covered stent through an FDA approved clinical trial (COAST II) should be considered. In addition, caution is advised for patients with a connective tissue disorder, blood stream infection, or inflammatory aortitis. Pregnancy is also considered a contraindication due to the radiation exposure to the developing fetus.

Pulmonary arteriovenous malformation

The patient with diffuse small pulmonary AVM is not easily treated by transcatheter closure without significant loss to normal lung function.

Coronary artery fistulae

Mainly if the anatomic location is not conducive with positioning the delivery catheter.

Details of how the procedure is performed

Patent ductus arteriosus

Femoral artery and femoral venous sheaths. Angiography is taken from the proximal descending aorta or from the ductal vessel entrance from the aorta. A duct occluder size is chosen to position within the ductus and moderately compresses the duct device. The device is positioned across the PDA from the venous access site and deployed within the PDA. Repeat angiography from the aorta prior to release. Release of the device.

Coarctation of the aorta

The procedure is performed with the patient either sedated or anesthetized using a transfemoral venous and arterial access. Heparin 5,000 units is administered and monitored to maintain an ACT >250 seconds.

A standard right heart catheterization and shunt calculations are performed to obtain the cardiac output and assess for comorbidities. Either, simultaneous, or “pull-back” ascending and descending aortic pressure measurements determine the gradient across the coarctation segment, location of the obstruction, and then angiography demonstrates aortic anatomy.

Diameter measurements to obtain:

  • Ascending aortic maximum

  • Proximal transverse aortic arch (just beyond the origin of the innominate artery)

  • Distal transverse aortic arch (at the origin of the last brachiocephalic artery)

  • Aortic isthmus (diameter of aorta beyond the origin of the last brachiocephalic artery)

  • Coarctation minimum diameter

  • Coarctation length

  • Postcoarctation aortic maximum

  • Aorta at the level of the diaphragm

  • Stent Implantation

The stent implantation balloon diameter should be no more than 33% greater than the minimum diameter of the "test dilation balloon" and not greater than 1.1 times the lesser diameter of the distal transverse arch or the descending aorta at the level of the diaphragm. The length of the stent selected should be adequate to cover the coarctation segment and in general is usually at least 36 mm.

Use of right ventricular pacing or other techniques to help prevent distal stent embolization are helpful for transverse arch interventions but generally are not necessary for isthmus coarctation. Both the bare metal and covered Cheatham Platinum (CP) stents can be deployed on a BiB (balloon-in-balloon) catheter to allow precise positioning of the stent.

Pulmonary arteriovenous malformation

Transfemoral or jugular vein approach. Placement of a long sheath in the branch pulmonary artery and subsequent use of angled catheters to locate the entry or entrances to the AVM. Then a guide catheter is positioned within the AMV to place the Amplatzer device (Amplatzer vascular plug or duct occluder, of VSD device).

Coronary artery fistulae

Transfemoral or radial artery approach. Placement of a guide catheter in the ascending aorta and subsequent use of angled catheters to locate the entrances to the coronary fistulae. Then a guide catheter is positioned within the coronary artery fistulae to place the Amplatzer vascular plug or coil.

Outcomes (applies only to therapeutic procedures)

Patent ductus arteriosus

Amplatzer duct occluder use was first reported in 1998 by Masura et al, and the device received FDA approval in May 2003 following the results of a multicenter trial. The ADO was designed for easy implantation into moderate to large sized PDAs with lower risk of embolization and residual leak. In the multicenter trial, the device proved to be very effective; 435 devices were successfully placed in 439 patients and 428 had complete ductal closure (98%). ADO use was also safe as major complications occurred in only 2.3% of patients, including two device embolizations and one patient death.

Coarctation of the aorta

The goal with stent angioplasty is to have zero or less than a 5 mm Hg gradient after stent placement. If there remains >5 mm Hg peak systolic pressure gradient across the stented region in the presence of no aortic wall injury, additional stent dilation can be considered.

Pulmonary arteriovenous malformation

Angiography is taken in the branch pulmonary artery or microcavitation injection is given in the branch pulmonary artery with transesophageal or intracardiac echocardiography to determine if there is any residual shunting to the left atrium from the pulmonary arteries. In addition, the systemic saturations will increase if the AMV shunt burden is large.

Coronary artery fistulae

Most coronary artery fistulae are small, and patients are asymptomatic as myocardial blood flow is not compromised. A continuous murmur may be audible at the left lower sternal border. With increased flow, the coronary artery branches proximal to the shunt site become significantly enlarged.

If untreated, fistulae cause clinical symptoms in about 20% of patients less than 20 years of age and in 63% of patients older than 20 years of age. Symptoms and sequelae include chronic myocardial ischemia and angina, congestive heart failure, cardiomyopathy, myocardial infarction, pulmonary hypertension, endocarditis, and rarely fistula rupture.

Alternative and/or additional procedures to consider

Patent ductus arteriosus

Surgical closure of PDA, first performed in 1938, has been replaced over the last decade by transcatheter closure devices in both children and adults.

Coarctation of the aorta

Surgical reconstruction of the aorta was the treatment from 1945 until the 1980s, when balloon angioplasty became a competitive option. More recently endovascular stents for both native and recurrent coarctation of the aorta have gained acceptance as the procedure of choice in teenagers and young adults who have achieved full growth. While early and midterm follow-up data appear to favor stent angioplasty over balloon dilation in adults, it is important to note that stenting the aorta may still be associated with serious adverse events. Long-term outcomes remain unknown.

Covered stents may become primary therapy in the future in an effort to avoid potential complications of arterial dissection, tear, or rupture, but are not yet available in the United States in sizes large enough for a typical aorta. There are no comparative studies yet comparing covered stents with other options.

Pulmonary arteriovenous malformation

Surgical lung resection for pulmonary AVM is rarely required.

Coronary artery fistulae

Surgical ligation is usually performed on a beating heart from the epicardial surface, is associated with low mortality and morbidity, and has excellent immediate and long-term outcomes.

Complications and their management

Serious adverse event and management

Patent ductus arteriosus

Device embolization during PDA closure is 0.4% to 1.4% in larger series reported in the literature; smaller series had higher rates. Although rare, the event can be associated with emergent surgery and death.

Coarctation of the aorta

Potential serious adverse events:

  • Stroke or transient ischemic attack

  • Myocardial infarction

  • Aortic rupture

  • Aortic dissection with distal obstruction

  • Femoral artery injury requiring either thrombolytic therapy or surgical arterioplasty

  • Paradoxical hypertension requiring ICU management with intravenous medications

  • Aortic aneurysm, requiring catheter-based or surgical intervention

  • Stent dislodgement or embolization producing aortic side branch obstruction or requiring surgical intervention

  • Stent fracture leading to loss of structural integrity

  • Restenosis, requiring reintervention

Pulmonary arteriovenous malformation

Paradoxical embolic events or device/coil/material embolization.

Coronary artery fistulae

Thrombosis or occlusion of the coronary artery treated with anticoagulation, TPA, or transcoronary stent angioplasty.

What’s the evidence?

Patent ductus arteriosus

This is the key studies for transcatheter interventions for patent ductus arteriosus

Pass, RH, Hijazi, ZM, Hsu, DT, Lewis, V, Hellenbrand, WE. "Multicenter USA Amplatzer Patent Ductus Arteriosus Occlusion Device Trial. Initial and One Year Results". J Am Coll Cardiol. vol. 44. 2004. pp. 513-519.

This article summarizes the multicenter results for the Amplatzer patent ductus arteriosus occluder device.)

Additional studies for transcatheter interventions for patent ductus arteriosus:

Coggin, CJ, Parker, KR, Keith, JD. "Natural history of isolated patent ductus arteriosus and the effect of surgical correction: twenty years' experience at The Hospital for Sick Children, Toronto". Can Med Assoc J. vol. 102. 1970. pp. 718-20.

(This article reviews the natural history for patients after surgical patent ductus arteriosus ligation.)

Masura, J, Walsh, KP, Thanopoulos, B, Chan, C, Bass, J, Goussous, Y, Gavora, P, Hijazi, ZM. "Catheter closure of moderate-to large-sized patent ductus arteriosus using the new Amplatzer duct occluder: Immediate and short-term results". J Am Coll Cardiol. vol. 31. 1998. pp. 878-82.

(This article reviews the results or Amplatzer PDA occlusion for larger patent ductus arteriosus.)

Faella, HJ, Hijazi, ZM. "Closure of the patent ductus arteriosus with the Amplatzer PDA Device: Immediate results of the International clinical trial". Cathet Cardiovasc Intervent. vol. 51. 2000. pp. 50-54.

(This article reviews the results or Amplatzer PDA occlusion for patent ductus arteriosus.)

Coarctation of the aorta

These are the key studies for transcatheter interventions for coarctation of the aorta:

Cheatham, JP. "Stenting of Coarctation of the Aorta". Catheter Cardiovasc Interv. vol. 54. 2001. pp. 112-125.

(This is a nice review article for stent angioplasty of coarctation of the aorta.)

Mahadevan, VS, Vondermuhll, IF, Mullen, MJ. "Endovascular aortic coarctation stenting in adolescents and adults: Angiographic and hemodynamic outcomes". Catheter Cardiovasc Interv. vol. 67. 2006. pp. 268-275.

Additional studies for transcatheter interventions for coarctation of the aorta:

Qureshi, SA, Zubrzycka, M, Brzezinska-Rajszys, G. "Use of covered Cheatham-Platinum stents in aortic coarctation and recoarctation". Cardiol Young. 2004. pp. 50-4.

(This article reviews the use of covered stents for coarctation of the aorta.)

Tzifa, A, Ewert, P, Brzezinska-Rajszys, G. "Covered Cheatham-platinum stents for aortic coarctation: early and intermediate-term results". J Am Coll Cardiol.. vol. 47. 2006 Apr 4. pp. 1457-63.

(This article reviews the use of covered stents for coarctation of the aorta.)

MacDonald, S, Thomas, SM, Cleveland, TJ, Gaines, PA. "Angioplasty or stenting in adult coarctation of the aorta? A retrospective single center analysis over a decade". Cardiovasc Intervent Radiol. vol. 26. 2003. pp. 357-64.

Fawzy, ME, Awad, M, Hassan, W. "Long-term outcome (up to 15 yrs) of balloon angioplasty of discrete native coarctation of the aorta in Adolescents and adults". J Am Coll Cardiol. vol. 43. 2004. pp. 1062-7.

(This article presents long term outcome for stent angioplasty of coarctation of the aorta.)

Pulmonary arteriovenous malformation

These are the key studies for transcatheter interventions for pulmonary arteriovenous malformations:

White, RI, Lynch-Nylan, A, Terry, P. "Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy". Radiology. vol. 169. 1988. pp. 663-9.

(This article reviews different techniques for transcatheter closure of pulmonary arteriovenous malformations.)

Swanson, KL, Prakash, UBS, Stanson, AW. "Pulmonary arteriovenous fistulas: Mayo clinic experience, 1982-1997". Mayo Clin Proc. vol. 74. 1999. pp. 671-80.

(This article reviews the Mayo Clinic experience for transcatheter closure of pulmonary arteriovenous malformations.)

Additional studies for transcatheter interventions for pulmonary arteriovenous malformations:

Post, MC, Letteboer, TG, Mager, JJ, Plokker, TH, Kelder, JC, Westermann, CJ. "A pulmonary right-to-left shunt in patients with hereditary hemorrhagic telangiectasia is associated with an increased prevalence of migraine". Chest. vol. 128. 2005. pp. 2485-9.

(This article reviews pulmonary arteriovenous malformations and its association with migraine headaches.)

Post, MC, Thijs, V, Schonewille, WJ. "Embolization of pulmonary arteriovenous malformations and decrease in prevalence of migraine". Neurology. vol. 66. 2006. pp. 202-5.

(This article reviews pulmonary arteriovenous malformations closure and how the migraine headache burden is reduced.)

Baldi, S, Rostagno, RD, Zander, T, Rabellino, M, Maynar, M. "Occlusion of a pulmonary arteriovenous fistula with an Amplatzer vascular plug". Arch Bronconeumol. vol. 43. 2007. pp. 239-41.

(This article reviews pulmonary arteriovenous malformations closure using the Amplatzer vascular plug device.)

Coronary artery fistulae

This is the key study for transcatheter coronary artery fistulae closure:

Armsby, LR, Keane, JF, Sherwood, MC. "Management of coronary artery fistulae. Patient selection and results of transcatheter closure". J Am Coll Cardiol. vol. 39. 2002. pp. 1026.

(This article reviews which patient should undergo coronary artery fistulae closure and the results.)

Additional studies for transcatheter coronary artery fistulae closure:

Liberthson, RR, Sagar, K, Berkoben, JP. "Congenital coronary arteriovenous fistula". Report of 13 patients, review of the literature and delineation of management. Circulation. vol. 59. 1979. pp. 849.

(This article reviews how to manage patients with a coronary artery fistulae.)

Mavroudis, C, Backer, CL, Rocchini, AP, Muster, AJ, Gevitz, M. "Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization". Ann Thorac Surg. vol. 63. 1997. pp. 1235-42.

(This article reviews which patient should undergo coronary artery fistulae closure and the results.)

Reidy, JF, Anjos, RT, Qureshi, SA. "Transcatheter embolization in the treatment of coronary artery fistulas". J Am Coll Cardiol. vol. 18. 1991. pp. 187.

Hakim, F, Madani, A, Goussous, Y. "Transcatheter closure of a large coronary arteriovenous fistula using the new Amplatzer duct occluder". Cathet Cardiovasc Diagn. vol. 45. 1998. pp. 155.

(This article reviews transcatheter coronary artery fistulae closure using the Amplatzer duct occluder device.)

Qureshi, SA, Zubrzycka, M, Brzezinska-Rajszys, G. "Use of covered Cheatham-Platinum stents in aortic coarctation and recoarctation". Cardiol Young. 2004. pp. 50-4.

(This article reviews the use of covered stents for coarctation of the aorta.)

Tzifa, A, Ewert, P, Brzezinska-Rajszys, G. "Covered Cheatham-Platinum stents for aortic coarctation: early and intermediate-term results". J Am Coll Cardiol. vol. 47. 2006. pp. 1457-63.

(This article reviews the use of covered stents for coarctation of the aorta.)

MacDonald, S, Thomas, SM, Cleveland, TJ, Gaines, PA. "Angioplasty or stenting in adult coarctation ofthe aorta? A retrospective single center analysis over a decade". Cardiovasc Intervent Radiol. vol. 26. 2003. pp. 357-64.

Fawzy, ME, Awad, M, Hassan, W. "Long-term outcome (up to 15 yrs) of balloon angioplasty ofdiscrete native coarctation of the aorta in adolescents and adults". J AmColl Cardiol. vol. 43. 2004. pp. 1062-7.

(This article presents long-term outcome for stent angioplasty of coarctation of the aorta.)

Pass, RH, Hijazi, ZM, Hsu, DT, Lewis, V, Hellenbrand, WE. "Multicenter USA Amplatzer Patent Ductus Arteriosus Occlusion Device Trial. Initial and One Year Results". J Am Coll Cardiol. vol. 44. 2004. pp. 513-519.

(This article summarizes the multicenter results for the Amplatzer patent ductus arteriosus occluder device.)

Additional studies for transcatheter interventions for patent ductus arteriosus:

Coggin, CJ, Parker, KR, Keith, JD. "Natural history of isolated patent ductus arteriosus and theeffect of surgical correction: twenty years' experience at The Hospitalfor Sick Children, Toronto". Can Med Assoc J.. vol. 102. 1970 Apr 11. pp. 718-20.

(This article reviews the natural history for patients after surgical patent ductus arteriosus ligation.)

Masura, J, Walsh, KP, Thanopoulos, B, Chan, C, Bass, J, Goussous, Y, Gavora, P, Hijazi, ZM. "Catheter closure of moderate-to large-sized patent ductusarteriosus using the new Amplatzer duct occluder: Immediate andshort-term results". J Am Coll Cardiol. vol. 31. 1998. pp. 878-82.

(This article reviews the results or Amplatzer PDA occlusion for larger patent ductus arteriosus.)

Faella, HJ, Hijazi, ZM. "Closure of the patent ductus arteriosus with the Amplatzer PDA Device:Immediate results of the International clinical trial". Cathet CardiovascIntervent. vol. 51. 2000. pp. 50-54.

(This article reviews the results or Amplatzer PDA occlusion for patent ductus arteriosus.)
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