2020

Unicuspid aortic valve repair with bicuspidization in the paediatric population

Unicuspid aortic valve repair with bicuspidization in the paediatric population.   Matsushima S, Heß A, Lämmerzahl JR, Karliova I, Abdul-Khaliq H, Schäfers HJ.   Eur J Cardiothorac Surg. 2020 Oct 1:ezaa285. doi: 10.1093/ejcts/ezaa285. Online ahead of print.   Take Home Points:   Unicuspid aortic valve repair in pediatric patients remains a significant challenge. Bicuspidization is a technique used for repair of unicuspid aortic valves, involving the creation of a second functional commissure using patch material. The technique of bicuspidization appear safe and reproducible in experienced hands, with excellent survival and good freedom from re-intervention.     Commentary from Dr. Luis Quinonez (Boston MA. USA), congenital heart surgery section editor of Journal Watch: This is a review of 60 consecutive patients, ages 1 through 18 years (median 13), with unicuspid aortic valves who underwent aortic valve repair for stenosis, regurgitation, or a combination. Half the patients had undergone a previous valvuloplasties. The repair technique is bicuspidization. The technique involves creation of a second functional commissure opposite the best developed commissure using a combination of detached autologous valve tissue and patch material. The new commissure is created at the same height as the existing one. The concepts of geometric height and effective height are described, where the effective height should be approximately 50% of the geometric height. The geometric height is curved the length of the leaflet from base to free edge and the effective height is the vertical distance between the annulus and free edge. The study spans between 2003-2018 and uses various patch materials and an external suture annuloplasty. Overall survival was 96% at 10 years. Freedom from aortic valve reoperation was 73% at 5 years and 50% at 10 years. The time to reoperation was 0.2 to 13 years (median 5.2).   Significance: Durable repair of unicuspid aortic valves remains a significant challenge in the pediatric population. Replacement options may be limited in younger age groups.   Comment: This reports presents a sizable experience with repair of unicuspid aortic valves in the pediatric population. The technique of bicuspidization has been used by Dr. Schafer’s group in Germany since 2003. It is certain it has gone through several modifications over time. The technique is not described in sufficient detail to apply. Although the age range of the patient's is wide it is notable that half the patients were under 13 years of age and that 12 of the 60 patients were between 1 and 5 years of age, suggesting the technique can be applied early on even in small annuli. The authors mention that the technique can be applied to an aortic root size is down to 10 mm. Survival for the overall cohort was excellent. The freedom from aortic valve reoperation is very acceptable. Although there were some early failures, the median time of re-intervention was just over 5 years, which would allow enough growth to expand the options for re-repair or replacement. When looking at the patch material used, Gore-Tex performed the worst. Interestingly, fixed autologous pericardium did not perform as well as the other decellularized patches, although the follow-up for the latter is less than 5 years. The results of this study support the notion that in experienced hands unicuspid aortic valve repair can be effectively used "as a bridge to more definitive AVR". The question remains whether the technique is sufficiently standardized to make it reproducible in less experienced hands.      

READMORE

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.   Dorobantu DM, Sharabiani MTA, Taliotis D, Parry AJ, Tulloh RMR, Bentham JR, Caputo M, van Doorn C, Stoica SC.Eur J Cardiothorac Surg. 2020 Oct 1;58(4):825-831. doi: 10.1093/ejcts/ezaa069.PMID: 32187367   Corrigendum to 'Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database' [Eur J Cardiothorac Surg 2020;58:825-31].   Dorobantu DM, Sharabiani MTA, Taliotis D, Parry AJ, Tulloh RMR, Bentham JR, Caputo M, van Doorn C, Stoica SC.Eur J Cardiothorac Surg. 2020 Oct 1;58(4):873. doi: 10.1093/ejcts/ezaa190.PMID: 32688385 No abstract available.     Commentary from Dr. Frederic Jacques (Quebec City, Quebec, Canada), congenital heart surgery section editor of Journal Watch: Dorobantu et al. published a study in the European Journal of Cardiothoracic Surgery trying to answer the question: Does age at the time of the first pulmonary valve replacement (PVR) among repaired tetralogy of Fallot (ToF) patients impact the long-term outcomes?[1] More specifically, it addresses survival. In this national registry-based multicentric retrospective study, the authors included all (707) ToF patients aged 15 or older who had to have PVR in the UK. They set to determine an age at which patients were more likely to survive both the procedure and in the long-term. They used a Cox regression model as well as CART (classification and regression tree analysis) to identify the optimal cut-off age. Both methods identified 35 years old as their cut-off, with older patients experiencing a 5.6-fold increased risk of dying at 10 years after PVR compared to the younger patients. Said differently, patients under 35 years of age at first PVR had a 10-year mortality of 1.3% compared to 10.4% for those between 35 and 50 years. Older patients required more tricuspid repair or replacement (15% vs. 6%) and arrhythmia surgery (18% vs. 6%) compared to the younger patients. The association of tricuspid valve surgery was linked to a worse survival in those between 35 and 50 years old, but not for the younger patients. Both age groups had worse survival than the general UK population, but the mortality excess was less for those with PVR under 35 (0.9%) compared to the 35-50 age group (7.7%).   Significance: Current guidelines for PVR among ToF patients do not take age into account in setting the surgical indication and timing but they likely should include guidance on this point. This study raises the question: Should we operate on patients earlier to achieve better long-term outcome?   Comment: With this type of registry, surgical indication criteria are not strongly defined, but one can reasonably assume most patients were treated with current Western standards. The most important strength of this study is that it is based on the mandatory inclusion of all the congenital patients from one country: no patient is missed. This likely results in catching subclinical right-sided damage that have not reach clinical significance. The reader is well aware that both residual regurgitation and stenosis are linked to myocardial damage, impact on exercise tolerance and even survival. Current guidelines recommend PVR when either volume or pressure overload had resulted in clinical signs and symptoms. So far, the literature has shown repercussion of PVR on RV volume and symptoms, but clear survival benefit is still to be demonstrated. Recent findings suggest that most of the hemodynamic recovery is seen immediately after surgery. This supports the idea that once right ventricular damage has been severe enough and long-standing, it might not be reversible. This also favors earlier intervention, since relieving the burden on the right ventricle earlier may prevent other comorbidities from developing. This study shows that older patients had more tricuspid regurgitation and arrhythmias. Obviously, a spectrum of subclinical myocardial damage occurs in the background before reaching to other clinically significant lesions. It is not known what portion of this damage is reversible. The authors suggest that current guidelines should be revisited not only based on hemodynamic parameters and symptoms, but that age should be accounted for in order to achieve a survival that approaches the general population as closely as possible. Will the paradigm shift from reacting to hemodynamic complications to preventing them?    

READMORE

Use of a dilatable exGraft™ conduit in single ventricle palliation

Use of a dilatable exGraft™ conduit in single ventricle palliation. Qadir A, Tannous P, Stephens EH, Kalra A, Forbess JM, Nugent A. Ann Thorac Surg. 2020 Jan 25. pii: S0003-4975(20)30075-8. doi: 10.1016/j.athoracsur.2019.12.024. [Epub ahead of print] PMID: 31991132 Similar articles Select item 31982878   Take Home Points: The major limitation of conduits made with prosthetic material is that they do not dilate much or grow with the patient. The development of a novel, balloon-expandable ePTFE conduit may change the management of patients that require a prosthetic conduit. This is the first published case of this conduit. Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch: Surgically-placed conduits and vascular grafts are commonplace in congenital heart surgery. One obvious limitation to placing a prosthetic conduit between a ventricle and great vessel (as opposed to a homograft or autograft) is the lack of growth potential. A prosthetic conduit that could be dilated could revolutionize the field, as it would add the ability to minimize or delay redo surgeries, thus decreasing the associated morbidity and mortality. A start-up company in Pittsburgh (PECA labs) has developed a novel version of the ePTFE graft that has been shown to be dilated up to 200% its original diameter in bench trials. I chose to highlight this case report because of its novelty. As a disclaimer, I am an unpaid consultant for this company and have had the ability to collaborate with this group of researchers on animal studies, some of which are on-going. Thus, I can attest first-hand that the graft handles well and does indeed dilate without significant recoil months after implantation. The graft also has the novel advantage of having radiopaque markers on it to allow for safer dilation during catheterization (Figure 1). As mentioned in this case report, the graft is FDA-approved and available for surgical implantation in human beings. This report represents the first published case of implantation and dilation of the novel exGraft™ in a patient undergoing congenital heart surgery. The case report describes a relatively standard Stage 1 palliation of a baby born with hypoplastic left heart syndrome (MA/AA). The surgeon used of a 6 mm exGraft™ as an RV-PA (Sano) conduit in lieu of the relatively standard ring-enforced PTFE graft that is often used for Sano construction. Aspirin alone was used for shunt thrombosis prophylaxis. The patient underwent a pre-Glenn catheterization at age 5 months. A gradient across the conduit was found to be 60-65 mmHg and the conduit was then dilated to 9 mm by serial dilations (Figure 2). There was mild residual narrowing at the insertion site into the pulmonary arteries, but otherwise the conduit held its dilated diameter and did not require a stent placement. The patient proceeded to a Glenn at 7 months of age that had an uncomplicated postoperative course. At the time of the explant of the conduit, it had maintained its internal diameter 2 months after dilation (Figure 3). As stated, this is the first published application of the novel exGraft™ in congenital heart surgery. The safe dilation of the graft to 150% its original diameter in this patient represents a potential milestone for our field. The use of the exGraft™ as a Sano conduit during a stage 1 palliation represents one of many potential uses of this graft. There is a range of sizes that would allow the graft to be used as a modified BT-shunt, interposition graft on large vessels, Fontan conduit and RV-PA conduits on larger children. The ability to implant and later enlarge a prosthetic graft would certainly decrease the morbidity and mortality associated with surgical correction of conduit dysfunction. The company is also working on valved-conduits that are balloon expandable, but those studies are still ongoing. In summary, the exGraft™ from PECA labs has the potential to change the paradigm of the timing and frequency of certain congenital heart surgical procedures.     Figure 1: Radiopaque markers on outer surface of an 8 mm exGraft Figure 2: In vivo dilation of the exGraft RV-PA conduit. A) Baseline angiogram demonstrating slight luminal irregularity and proximal stenosis of the exGraft. B) Stored fluoro image of maximum balloon dilation to 9 mm. (C and D) Final exGraft angiograms in lateral and caudal angulation showing interval increase in graft diameter. Figure 3: Images of explanted conduit. Note radio-opaque markers on outer surface of graft.

READMORE

Impact of trisomy 13 and 18 on airway anomalies and pulmonary complications after cardiac surgery.

Impact of trisomy 13 and 18 on airway anomalies and pulmonary complications after cardiac surgery. Swanson SK, Schumacher KR, Ohye RG, Zampi JD. J Thorac Cardiovasc Surg. 2020 Sep 1:S0022-5223(20)32473-9. doi: 10.1016/j.jtcvs.2020.08.082. Online ahead of print. PMID: 32981701   Take Home Points:   Cardiac surgery can be performed safely in the setting of Trisomy 13 and Trisomy 18. T13 and T18 patients undergoing cardiac operations have higher rates of airway complications and require longer ICU stays than matched controls. While operative interventions are likely to be successful, guidance for parents of these patients should include a discussion of postoperative respiratory support.     Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Trisomy 13 (Patau syndrome) and Trisomy 18 (Edwards syndrome) are both associated with high rates of congenital heart diseases, such as VSD, ASD and PDA.  More importantly, however, these chromosomal abnormalities are associated with a severely impaired long-term survival, with a median survival of less than 2 weeks.  As approximately 5-10% of patients survive over 1 year, it is perhaps not surprising that offering surgical treatment for cardiac malformations remains somewhat controversial.  However, as reports of long-term survival increase, more centers offer treatments, such as cardiac surgery, aimed at long-term survival.  Although multiple  have established that these patients can safely undergo surgery, fewer reports have described the postoperative courses of these patients, and the specific comorbidities which complicate postoperative recovery.  Because respiratory complications have been noted to be major complicators of recovery in previous series, this review was performed with a focus on identification and management of respiratory complications.  The authors performed a retrospective case-control study of all Trisomy 13 and Trisomy 18 (T 13/18) patients in a single center over 20 years undergoing cardiac surgery.  A 3:1 diagnosis-matched control group was identified, and individuals were further matched by age at surgery and then date of surgery.   In this study, 14 patients with T 13/18 were identified, and underwent surgical operations at a median age of 93 days. Trisomy 18 was more common than trisomy 13. The indications for operation  included VSD, coarctation, tetralogy of Fallot and complete AVSD.  64% of T 13/18 patients were admitted preoperatively , whereas only 13% of controls were.   Preoperatively, 3 of the T13/18 patients had airway evaluation by specialist physicians.  Patients with T13/18 were more likely to require preoperative respiratory support (71% vs 14%, P=0.001), including intubation and ventilation as well as noninvasive ventilatory assistance.   Operatively, there were no significant differences in cardiopulmonary bypass time, cross clamp time, or circulatory arrest time.   Postoperatively, 13 of the 14 T 13/18 patients experienced at least one complication, And all of these patients experienced at least one respiratory complication. Further, 58% of these patients had at least one non respiratory complication.  In addition to having higher rates of respiratory support preoperatively, T 13/18 patients also had high rates of post-operative respiratory complications, including prolonged mechanical ventilation prolonged noninvasive ventilation, reintubation , tracheostomy, and respiratory infections.   Some other notable complications, including infection (tracheitis and sepsis), need for gastrostomy tube placement, and increased need for vasoactive and inotropic support.   The authors conclude that although survival is good, hospital and ICU length of stay is longer in T13/18 patients than in matched controls.   This retrospective study demonstrates that those increases are at least correlated with an increased prevalence of respiratory and airway anomalies in these patients.   While not recommending against surgical repair for these patients, they suggest that awareness of these airway issue should be included in prenatal counseling, preoperative planning, and managing expectations for these patients.    

READMORE

Ross Procedures in Children With Previous Aortic Valve Surgery.

Ross Procedures in Children With Previous Aortic Valve Surgery. Buratto E, Wallace FRO, Fricke TA, Brink J, d'Udekem Y, Brizard CP, Konstantinov IE. J Am Coll Cardiol. 2020 Sep 29;76(13):1564-1573. doi: 10.1016/j.jacc.2020.07.058. PMID: 32972534   Take Home Points:   Initial Ross procedure in infancy may be associated with relatively higher mortality, justifying initial attempts to defer operation with aortic valve repairs, even if eventual replacement is necessary. Secondary Ross after initial valve repair is associated with superior long-term survival and freedom from autograft reoperation. Delaying the Ross operation by performing initial valve repair, when possible, may be preferred to initial Ross in infant patients.   Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Aortic valve disease requiring surgical repair in childhood can be a challenge, with limited options available for replacement.  Although the Ross procedure offers the best long-term freedom from reoperation in neonates and infants, the long term freedom from reintervention in some series remain disappointing, with high mortality rates in infants (16 – 22%).  Because of this, some centers advocate an approach that of initial aortic valve repair, whenever possible, followed by a secondary Ross procedure when necessary.  The goal of this retrospective analysis as to determine if there was a difference in outcome between the Ross operation when performed as a primary operation and when performed as a reoperation.   The authors retrospectively analyzed 541 aortic valve operations in their institution, 344 of which were aortic valve repairs, and 140 of which were Ross procedures.  68 (49%) of the Ross procedures took place after previous valve repairs, and 72 (51%) took place as the primary operation.   Patients undergoing a primary Ross were older (8.6 years vs 7 years), and had higher weights (28.9 kg vs. 19.7 kg) although these differences weren’t significant.  The early mortality was 5.6% (n = 4 of 72) in those undergoing primary Ross procedure, compared with 4.4% (n = 3 of 68) in those undergoing secondary Ross procedure.  This difference was not statistically significant (p = 1.0).  Freedom from reoperation at 10 and 15 years was 68.3% and 48.5% in those undergoing primary Ross procedure.  In those who underwent secondary Ross procedure, freedom from reoperation at 10 and 15 years was 62.2% and 47.0% respectively.  Again, this difference was not statistically significant.   In the  propensity-matched groups, however, survival at 10 and 15 years was 90.0% and 82.6% in the primary Ross procedure group, compared with 96.8% at both 10 and 15 years in the secondary Ross procedure group. In the propensity-matched analysis, this difference was statistically significant (p = 0.04).   The mechanisms for the improved survival and freedom from reoperation are discussed briefly, including possible stabilization of the autograft from postoperative scar and adhesion formation, but this was just postulated rather than investigated.  None were investigated in this retrospective review.   The authors concluded that secondary Ross procedure performed after initial aortic valve surgery achieves superior long-term survival and freedom from autograft reoperation compared with primary Ross procedure. A strategy of initial aortic valve surgery followed by delayed Ross procedure may provide better long-term survival and freedom from autograft reoperation in aortic valve disease in children.      

READMORE

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study View Article David M Kwiatkowski 1, Christopher W Mastropietro 2, Katherine Cashen 3, Saurabh Chiwane 4, Saul Flores 5, Ilias Iliopoulos 6, Karan B Karki 7, Karl Migally 8, Monique R Radman 9, Christine M Riley 10, Peter Sassalos 11, Jennifer Smerling 12, John M Costello 13, Collaborative Research from the Pediatric Cardiac Intensive Care Society Investigators PMID: 32858217;  DOI: 10.1053/j.semtcvs.2020.08.014 Take Home Points 29% of patients with ALCAPA in this large, retrospective, multicenter cohort study presented at greater than one year of age. Patients presenting at >1 year of age had excellent intermediate term survival (no mortalities in this series.) 11% of patients presenting with ALCAPA at greater than 1 year of age presented with cardiac arrest. Although severe LV dysfunction was less common for patients presenting at greater than 1 year than for those presenting at less than 1-year, chronic arrythmia and mitral regurgitation were common. Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Although most patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) present with heart failure or shock in the first months of life, a population of patients who develop extensive collateralization do not present with acute myocardial ischemia.  Most previous studies of this population are small, and limited to single center studies.  The authors of this study sought to characterize the clinical presentation, outcomes of patients who underwent ALCAPA repair older than 1 year of age with the collective experience from 21 congenital heart centers over 10 years. They hypothesized that this older cohort would present with different clinical symptoms and have different postoperative outcomes than a younger cohort. A multicenter retrospective cohort study including all patients who underwent initial coronary artery surgery for ALCAPA between January 2009 and March 2018 (21 centers; 248 patients).  Patients who underwent surgical correction at an age > 365 days were identified.  Patients with ARCAPA, single coronary, or other significant cardiac lesions were excluded.  Of the 248 patients identified, 72 underwent operation at greater than 1 year of age.  The median age in this group was 8.3 years, 15 of whom were older than 18.  There was no correlation between the degree of LV dysfunction, and the severity of mitral regurgitation.  11% presented with cardiac arrest, and 4% presented in cardiogenic shock.  35% of patients had been admitted to the hospital for another diagnosis, and 44% of these patients were discharged without the diagnosis of ALCAPA being made.  Compared to the younger patients, patients in the older cohort were more likely to present after cardiac arrest, but less likely to present with respiratory failure or shock, and less likely to require preoperative inotropic or ventilatory support.   No patients received transplantation during the study, although one was listed.  At 1-year follow-up, only 5% had moderate or worse systolic dysfunction, and 19% had moderate or worse mitral regurgitation.  Among the 13 patients who presented with moderate or worse LV systolic dysfunction, recovery was seen in 15% by discharge, 89% by 1-year and 90% by 3-year follow-up. The authors concluded that patients who presented with ALCAPA older than 1 year of age were more likely to present with cardiac arrest than the younger cohort, the majority of patients who presented older did not have ventricular dysfunction, MR or LV dilation.  Further, they confirmed that outcome of repair after 1 year of age is associated with good intermediate term outcome, and recommend continued follow-up to assess long term outcome. Table 1.

READMORE

Risk Factors for Failed Fontan Procedure Following Stage 2 Palliation

Risk Factors for Failed Fontan Procedure Following Stage 2 Palliation View Article Masamichi Ono 1, Melchior Burri 2, Benedikt Mayr 3, Lisa Anderl 4, Martina Strbad 4, Julie Cleuziou 4, Alfred Hager 5, Jürgen Hörer 4, Rüdiger Lange 6 PMID: 32828751;  DOI: 10.1016/j.athoracsur.2020.06.030 Take-Home Points Retrospective, single center study, retrospectively analyzing 525 patients receiving Stage 2 Palliation (S2P) with superior cavopulmonary connection for single ventricle palliation over 20 years. The incidence of Fontan completion following S2P was, 83.9% at 3 years and 87.1% at 5 years. HLHS, UAVSD, reduced EF, increased EDP and increased PA pressure were all associated with inability to tolerate S2P. Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  The authors analyzed the results of two decades worth of bidirectional superior cavopulmonary (Glenn) shunts (BCPS) in order to understand risk factors that may contribute to failure to complete total cavopulmonary connection.  They reviewed 525 patients undergoing BCPS over 20 years in a single institution. Specifically, they were interested in identifying if there were anatomic, physiologic, or hemodynamic scenarios that correlated with inability to reach successful Fontan circulation. A competitive risk analysis was performed between one of three states: Fontan completion, death, and being alive without Fontan.   Overall, the incidence of Fontan completion was good, 83.9% at 3 years and 87.1% at 5 years.  The overall mortality was 10.4% at 3 years and 10.7% at 5 years, with a relatively small number (5.7% and 2.2% at 3 and 5 years respectively) being alive and not having completed a Fontan.  Risk factor analysis was performed by first completing a Cox regression model, and on univariate analysis, HLHS, UAVSD, dominant RV, significant AVV regurgitation requiring an AVV procedure, higher pulmonary artery pressure (PAP), higher left atrial pressure, elevated ventricular EDP and reduced ventricular ejection fraction were associated with those patients who had not completed a Fontan procedure.   On multivariate analysis, HLHS (HR = 4.1), UAVSD (HR 10.1), higher PAP, and reduced function (HR 4.2) emerged as independent risk factors.   AV valve regurgitation requiring AV valve repair was not an independent risk factor in all patients, however, the need for an AV valve repair was significantly higher in patients with UAVSD. The authors conclude that stage 2 palliation is low risk, and leads to excellent rates of Fontan completion, but that some patient groups do have increased risk of complications, mortality, and inability to achieve Fontan circulation.  These are, perhaps not surprisingly, anatomic diagnoses of HLHS and UAVSD, impaired ventricular function as assessed by echocardiography or elevated filling pressures, and elevated pulmonary artery pressure.   An examination of how each of these factors contribute to failure to progress, specific strategies to mitigate them, or a comparison of techniques applied to achieve stage 2 palliation, remains beyond the scope of this retrospective analysis.

READMORE

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.   Dorobantu DM, Sharabiani MTA, Taliotis D, Parry AJ, Tulloh RMR,...

read more

Routine Surveillance Catheterization is Useful in Guiding Management of Stable Fontan Patients. Patel ND, Sullivan PM, Sabati A, Hill A, Maedler-Kron C, Zhou S, Shillingford N, Williams R, Takao C, Badran S. Pediatr Cardiol. 2020 Jan 24. doi: 10.1007/s00246-020-02293-3. [Epub ahead of print] PMID: 31980851 Take Home Points: • Routine cardiac catheterization is a common part of Fontan surveillance algorithms. • Despite appearing to be clinically stable, a large proportion of Fontan patients in this cohort required transcatheter interventions; required initiation of pulmonary vasodilator therapy; and were identified to have hepatic fibrosis. Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: As survival of patients with single ventricle physiology palliated with a Fontan circulation improves it is becoming increasingly recognized that they are prone to developing significant end organ pathology. Various monitoring strategies have been proposed but all usually involve a surveillance catheterization 5-10 years after the Fontan surgery. The value of this assessment has not been fully explored. The authors sought to explore the utility of surveillance cardiac catheterization in stable Fontan patients at a single center. Sixty-three Fontan patients (mean age 14.6 ± 3.0 and mean time from Fontan surgery 11.3 ± 3.1 years) underwent cardiac catheterization over a 5-year period. The pre-catheterization echocardiograms (within a year) were unremarkable – 80% had normal ventricular function and less than mild AVV insufficiency. Baseline hemodynamics were mostly reassuring with 4 patients having a Fontan pressure > 15 mmHg and 19 having a PVRi > 2.0 iWu. Vasoreactivity testing with iNO was performed in 53 and half had a reduction in the PVRi of at least 20%. Fifty-seven patients underwent a trans-jugular liver biopsy at the time of procedure with more than half (32/57) demonstrating higher grades of congestive hepatic fibrosis. Forty percent of patients had an intervention at the time of the procedure: pulmonary artery stent (16), existing stent dilation (5), SVC angioplasty (4), Fontan stent (3), and others (7). There were no major complications. Despite the limitations of this work, the authors demonstrate that routine invasive assessment of Fontan patients can provide valuable information and allow for optimization of the Fontan circulation. Given the high morbidity experienced by this patient population it is important for providers to be pro-active in longitudinal Fontan assessment management as a stable Fontan is not necessarily a good Fontan

Routine Surveillance Catheterization is Useful in Guiding Management of Stable Fontan Patients. Patel ND, Sullivan PM, Sabati A, Hill A, Maedler-Kron C, Zhou S, Shillingford N, Williams R, Takao C, Badran S. Pediatr Cardiol. 2020 Jan 24. doi:...

read more