Fetal and Hybrid Procedures in Congenital Heart Diseases

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Identifying favorable anatomical variances including aneurismal tissue within the VSD, 17 along with newer device designs have helped reduce rates of heart block. Newer device design and application of softer existing devices has facilitated successful closure of primembranousVSDs with aortic valve prolapse Figure 2 and doubly committed sub-arterial VSDs. Ultimately investment in device design is necessary to develop lower profile delivery systems and device characteristics that will ensure device stability with closure rates similar to surgery and minimal trauma to the conduction system.

Series of images outlining retrograde perimembranous VSD closure via a left common carotid cutdown in a 6. A, B Patient positioning with feet pointing towards the head of the table to facilitate access to the left common carotid artery. C Insertion of 7 Fr short sheath following left common carotid cutdown. B Initial aortography demonstrating RCC prolapse white arrow. C Short axis TOE image confirming the defect. F Final colour Doppler TOE image in the short-axis confirming no residual leak across the ventricular septum. The great paradox of congenital heart disease therapy is the need to provide polar opposite interventions on the heart.

Whilst efforts are ongoing to provide a robust transcatheter alternative to surgical VSD closure, occasionally the need arises to create a VSD to decompress the left ventricle. Successful stenting of the ventricular septum following radiofrequency perforation has been reported, 19 although this is a complex and challenging intervention usually required in smaller infants and therefore not to be undertaken without careful consideration. Whether development of nonthermal, mechanical tissue fractionation using high-intensity ultrasound pulses, or histotripsy will evolve to provide a safer less invasive option for creation of both atrial and ventricular septal communications, remains to be seen.

Progression towards less invasive interventions in smaller infants is an inevitable marker of progress within the field of congenital heart disease. More promising outcomes have been seen with recent reports of transcatheter patent ductus arteriosus occlusion in extremely low birth weight infants, with procedural weights as low as g.

Stenting of the arterial duct has re-emerged, following initially disappointing outcomes, particularly since the description of the hybrid approach to hypoplastic left heart syndrome. Recent registry data would also suggest that procedure outcomes are less affected by volume of procedures with comparable results seen with low and high volume centers. Two large recent retrospective analyses comparing ductal stenting to surgical shunts have shown favorable outcomes in relation to post-procedural recovery and length of intensive care unit ICU stay, 27 as well as early and late survival, 28 in the ductal stented cohorts.

Again this has occurred in spite of the lack of a stent designed specifically for this purpose.

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Right ventricular outflow tract RVOT stenting in symptomatic infants with tetralogy of Fallot has also evolved to compete with surgical palliation in recent years, leading to shorter ICU and total hospital stay as well as better pulmonary artery growth. Efforts to preserve pulmonary valve function in this setting continue to provide a huge challenge to all involved in care of these patients.

Whether advances in transcatheter ablation techniques and equipment may resurrect attempts to remodel subpulmonic muscle bundles and promote growth of the pulmonary annulus, thus augmenting efforts to preserve pulmonary valve function, remains to be seen. Hybrid palliation, outside of those already mentioned above, include intraoperative pulmonary artery stenting, 33 vascular cutdowns for neonatal interventions, 34 as well as transcatheter pulmonary valve replacement.

Although most large congenital heart disease CHD treatment centers now include a hybrid catheterization laboratory, to date guidelines on appropriate layout for such a space and staffing requirements are lacking, as is consensus on appropriate lesions that may be treated in this way and strategies to do so. As the boundaries of reducing procedural mortality are tested, the consequences of smaller hemodynamic alterations become greater.

Significant advances have been made with newer intra-procedural imaging platforms focusing on radiation dose reduction, imaging quality and also advanced imaging modalities, fusing pre-procedural imaging with live acquisition images, or creating 3-D images through rotational angiography. Echocardiography-acquired images can be superimposed onto fluoroscopy images to guide complex closure procedures. Holography provides images created in real time from volumetric data, which float in the air during the procedure, in front of the operator and could provide an intuitive and interactive 3-D display for the interventionalist.

Early experience in a clinical setting has been reported, 40 however it is unclear as yet how this will translate into guiding interventional outcomes in a meaningful way. In the space of 15 years, transcatheter pulmonary valve replacement has evolved to become the procedure of choice to restore pulmonary valve function in appropriately sized dysfunctional right ventricular RV to pulmonary artery conduits. Growing experience with the procedure has lead to reports describing mounting of the pre-stent onto the valve system reducing the steps required for successful valve implantation, 45 and extension to patients with smaller RV-PA conduits and bioprsothetic valves.

Many dysfunctional RVOT's however remain too large for current balloon expandable systems and clinical experience with a number of self-expanding systems have been reported. Hybrid options for the dilated RVOT have also been reported to surgically reduce the size of the RVOT, 35 or provide simultaneous internal fillers during valve deployment via a subxiphoid approach. Series of images demonstrating transcatheter pulmonary valve replacement with Venus P valve in a patient with a previous transannular patch.

Non-surgical Alternatives in the Treatment of Congenital Heart Defects

B Balloon sizing demonstrated waisting at 33 mm. C Angiography with valve deployment within the left pulmonary artery. D Post valve deployment angiography in the main pulmonary artery confirming valvar competence. E En-face view of the valve demonstrating near circularity of the valve with no distortion of the aorta seen with aortic angiography. F Transthoracic echocardiography with color Doppler confirming no pulmonary incompetence. Transcatheter valve replacement in CHD patients has evolved beyond the pulmonary valve.

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The issue of how to deal with somatic growth remains unique to the CHD population. Although the properties of the Melody valve that facilitate valve function at a variety of diameters are attractive in the short-term, ultimately harnessing advances in tissue engineering to develop a valve with true growth potential has become one of the challenges for the future of this specialty. Congenital interventionalists have benefited from advances in other fields of medicine to provide an array of stent sizes and designs for various vessels, albeit none designed specifically for patients with congenital heart disease.

This has broadened our scope, however we remain limited by the challenge of somatic growth. Numerous approaches have been reported to deal with somatic outgrowth including high pressure balloon induced fracturing of small diameter stents, 55 or novel stent designs, consisting of 2 stent halves connected by reabsorbable sutures.

The benefits in this cohort of patients are arguably even greater than in adult patients particularly if restoration of vascular function can be achieved in the diseased vessel. Bioresorbable occluders particularly for the atrial septum are also attractive particularly with potential for increasing need to access the left atrium in later life.

Adequate data collection around congenital cardiac interventions has lagged behind adult cardiology and congenital cardiac surgery. Guidelines for interventions in adult congenital heart disease patients were included in a document, 61 with guidelines for interventions in pediatric patients published in However, providing guidelines is only part of the solution.

Many more decisions are involved in performing a specific intervention and efforts to reduce variation in practice with the introduction of a standardized clinical assessment and management plan SCAMP has been reported. We are all susceptible to a number of different subconscious traps and biases and it is inevitable that these decisions may impact delivery of care.

Finally, these registries are evolving to assess quality metrics in participating centers, providing benchmark goals for radiation doses for specific procedures amongst other outcomes variables.

There are a number of evolving interventions that are gaining traction at present including transcatheter creation of a reverse Potts shunt for patients with RV failure on the background of severe pulmonary hypertension Figure 4. Indeed magnetic resonance imaging MRI -guided creation of a superior cavopulmonary shunt has been described in a porcine model, 72 and one may wonder if future palliation of some single ventricle conditions may obviate the need for surgery at all and whether we will be performing these interventions without the need for ionizing radiation.

Series of images demonstrating transcatheter creation of a reverse Pott's shunt. A Initial simultaneous angiography in the descending aorta and main pulmonary artery confirming the proximity of the dilated left pulmonary artery to the descending aorta white arrows. B Deflectable sheath used to advance RF wire white arrow towards goose-neck snare in the pulmonary artery. C Repeat simultaneous angiography in the descending aorta and pulmonary artery demonstrating separation of the 2 vessels following RF perforation white arrows.


  • Congenital Heart Conditions & Treatments | Cedars-Sinai.
  • Fetal and Hybrid Procedures in Congenital Heart Diseases | Gianfranco Butera | Springer.
  • Non-Surgical Alternatives- Congenital Heart Defects?
  • Bibliographic Information.

E Pulmonary artery angiography demonstrating right-to-left shunt across the newly created reverse Pott's shunt white arrow. The future of our specialty will continue to be driven by the need to restore normal physiology with the least invasive means possible. As imaging modalities evolve, it will be possible to compute pre-procedurally variations in anatomical repair and determine the impact on flow patterns.

The impact of variations in inferior conduit anastomoses upon Fontan circuit efficiency has been reported. These variances in flow can be measured by MRI. A similar approach could be envisaged for septal defect closure with 3-D model printing of bespoke devices from a bioresorbable polymer for individual patients.

Hybrid Procedure - Avery's Story

Finally, as the field of transcatheter valve replacement continues to expand, focus will need to be directed towards the need for growth. Harnessing the broader medical interest in tissue engineering to patients with CHD, who may benefit most from this technology is paramount.

The basic concept is to create living material made by cellularized grafts that, once implanted into the heart, grows and remodels in parallel with the recipient organ Figure 5.

:: KCJ :: Korean Circulation Journal

Cartoon illustrating possible future strategies for the surgical management of newborns with CHD. If CHD is diagnosed prenatally, fetal cells may be harvested and iPS generated; as an alternative, umbilical cord stem cells can be isolated at the time of birth. When diagnosis of CHD is made after birth or in babies who require a palliative surgical operation soon after birth, stem cells may be isolated from surgical cardiac leftovers.

All these types of cells will allow the generation of a tissue-engineered graft endowed with growth and remodeling potential, necessary for the definitive correction of cardiac defects. Taken from Avolio et al. In summary, much has been achieved however, much more has yet to be realized. Historically, although at the forefront of innovation, congenital cardiac interventionalists have all too often been left to tail-coat on rapid advances made in adult interventions and left to improvise.

Dedicated device requires support from industry and academia with international device registries providing ongoing safety and efficacy data. Future potentials to extend the remit for minimally invasive therapies requires collaboration and continued development of a dedicated environment and tools to maximize optimal outcomes. Conflict of Interest: The author has no financial conflicts of interest. National Center for Biotechnology Information , U. Journal List Korean Circ J v. Korean Circ J. Published online Mar Damien Kenny , MD. Find articles by Damien Kenny.

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