ARCAPA in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

We report a case of anomalous origin of the right coronary artery from the pulmonary artery in a patient with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. The diagnosis was made during a proposed hybrid approach to stent the native right ventricular outflow tract, and an alternative surgical strategy was created.

Cardiac Rhabdomyomas Presenting with Critical Cardiac Obstruction in Neonates and Infants: Treatment Strategies and Outcome, A Single-Center Experience.

Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability. Surgical resection of the tumor, which has been the gold standard in alleviating obstruction, is not always possible and may be associated with significant morbidity and mortality. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be safe and effective in the treatment of TSC. We present the outcomes of neonates and an infant who received treatment for symptomatic rhabdomyomas at a tertiary cardiology center. Medical records were reviewed to obtain clinical, demographic, and outcome data. Six patients received interventions for symptomatic rhabdomyomas, median age at presentation was 1 day old (range from 1 to 121 days old), and 67% of the patients had a pathogenic mutation in TSC gene. One patient underwent surgical resection of solitary tumor at right ventricular outflow tract (RVOT) successfully. In the four patients with left ventricular outflow tract (LVOT) obstruction, two patients received combined therapy of surgical debulking of LVOT tumor, Stage I palliation procedure, and mTORi and two patients received mTORi therapy. One patient with RVOT obstruction underwent ductal stenting and received synergistic mTORi. Four of the five patients had good response to mTORi demonstrated by the rapid regression of rhabdomyoma size. 83% of patients are still alive at their latest follow-up, at two to eight years of age. One patient died on day 17 post-LVOT tumor resection and Hybrid stage one due to failure of hemostasis, in the background of familial factor VII deficiency. Treatment of symptomatic rhabdomyoma requires individualized treatment strategy based on the underlying pathophysiology, with involvement of multidisciplinary teams. mTORi is effective and safe in inducing rapid regression of rhabdomyomas. A standardized mTORi prescription and monitoring guide will ensure medication safety in neonates and infants with symptomatic cardiac rhabdomyoma. Although the majority of tumors responded to mTORi, some prove to be resistant. Further studies are warranted, ideally involving multiple international centers with a larger number of patients.

Multimodality imaging in delineation of complex sinus venosus defects and treatment outcomes over the last decade.

BACKGROUND: Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging. METHODS: Retrospective review of all patients from February 2008 to January 2019. RESULTS: Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5-15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6-15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter. CONCLUSION: Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.

Transcarotid Approach to Ventricular Septal Defect Closure in Small Infants.

Perimembranous ventricular septal defect closure in small infants has traditionally been a surgically treated defect, although alternative hybrid strategies are emerging. We aim to describe a novel approach to retrograde device closure of clinically relevant perimembranous ventricular septal defects in small infants via carotid cutdown. A retrospective review of all patients managed with attempted carotid cutdown for device closure of a perimembranous ventricular septal defect was recorded at a single tertiary cardiac centre. We summarized data on successful device deployment, conversion to open repair, complications, and length of stay. Eighteen infants with median (IQR) age of 7 months (5-9 months) and weight of 7.1 kg (6.5-7.8 kg) with clinically relevant PMVSD underwent attempted retrograde closure via carotid cutdown. Median (IQR) defect size was 8 mm (7-9 mm). Successful device deployment without significant aortic or tricuspid valve interference occurred in 15 (83%) patients. Three patients were converted to open repair, one following damage to the tricuspid valve apparatus. Median (IQR) hospital stay was 1 day (1-3 days). There were no complications related to carotid cutdown. Retrograde device closure of hemodynamically significant PMVSD is feasible and effective in small infants. Decision to convert to surgical repair should be made early if suboptimal device placement occurs. Carotid evaluation should be performed to rule out any access-related complications.

Right ventricular outflow tract stenting in symptomatic infants without the use of a long delivery sheath.

BACKGROUND AND OBJECTIVES: Right ventricular outflow tract (RVOT) stenting improves systemic oxygenation and facilitates pulmonary arterial growth in symptomatic infants prior to repair of tetralogy of Fallot. The aim of this study was to evaluate the safety and efficacy of RVOT stenting without the use of a long delivery sheath. METHODS: Retrospective data analysis of patients under 1 year of age undergoing RVOT stenting from January 2010 to January 2020 at a single tertiary pediatric cardiology center. RESULTS: Sixty-three RVOT stents were deployed during 53 procedures into 44 patients. The median age and weight at insertion were 41 days (range 2-204) and 3.6 kg (range 1.59-7) respectively. Thirty-one procedures were semi-elective and 22 were emergencies. Stent positioning was guided by transthoracic echocardiogram and/or RV angiography from a pigtail micro-catheter placed via the aorta. The median total procedure and fluoroscopy times were 67.5 (range 15-145) and 19 min (1-107), respectively. The median length of hospital stay was 7 days (range 1-258). Twenty-one patients were admitted to ICU post-procedure with a median ICU length of stay of 3 days (range 3-11). There were three major complications including two deaths within 30 days of the procedure. A patient with Cornelia de Lange Syndrome (1.8 kg) died following stent migration and inability to wean from emergency cardiopulmonary bypass and the second infant had an unexplained asystolic arrest post-procedure while awaiting transfer to ICU. CONCLUSIONS: RVOT stenting is technically possible with minimal complications without the need for a long delivery sheath. Additional imaging with transthoracic echocardiography can facilitate the safe deployment of the stent.

Hybrid Subxiphoid Perventricular Approach as an Alternative Access in Neonates and Small Children Undergoing Complex Congenital Heart Interventions.

The hybrid subxiphoid perventricular approach provides direct access through the heart and may alleviate the technical limitations of complex percutaneous interventions particularly in infants with low body weight. We present the outcomes from a tertiary cardiology center using this approach. We performed a retrospective review of all patients less than 15 kg who underwent a hybrid perventricular approach via a small subxiphoid incision. Medical records were reviewed to obtain clinical, demographic and outcome data. Seventeen patients underwent 18 hybrid perventricular procedures using a subxiphoid approach. Median age at time of procedure was 4.6 months (IQR = 1.6 to 18 months) and median weight was 6.2 kgs (IQR = 3.4 to 8.6 kgs). Six patients underwent hybrid pulmonary valve replacement (PVR), 5 patients underwent pulmonary outflow stenting, and 5 infants underwent hybrid ventricular septal defect (VSD) device closure. One patient with a single ventricle who did not tolerate a percutaneous approach underwent left pulmonary artery (LPA) stenting for severe LPA coarctation with subsequent right ventricular outflow tract (RVOT) stenting. One further patient underwent implantation of a larger diameter stent for pulmonary artery bifurcation stenosis. Procedure success rate was 89% with two of the VSD cases reverted to open surgical repair. There were no intra-procedural complications; however, one patient died within 72 h. Minor adverse events occurred in 2 patients including a wound infection in one patient with an immunodeficiency syndrome. Hybrid subxiphoid perventricular approach provides an excellent alternative access to the heart especially in low birth weight infants to prevent hemodynamic instability or in small children requiring large delivery sheaths.

Procedural technique for hybrid pulmonary valve replacement in infants and small children.

OBJECTIVES: Hybrid approach to pulmonary valve replacement (PVR) in the paediatric population has been reported, although data in infants and small children are limited. Several strategies are now possible. The aim of this study is to review our hybrid PVR strategy in a complex patient cohort, outlining a variety of approaches employed in our centre. METHODS: We performed a retrospective review of infants and small children who underwent hybrid PVR between May 2017 and April 2019 in a single tertiary cardiology centre. Medical records were reviewed to ascertain demographic, clinical and outcome data. RESULTS: Ten patients with a median (interquartile range) age of 1.5 years (1.1-1.9) and weight of 8.8 kg (8-10.6) were managed with hybrid pulmonary valve insertion. Eight patients had perventricular approach (4 sternotomy and 4 subxiphoid) and 2 patients had surgically sutured valve. Six patients underwent cardiopulmonary bypass for associated lesions. Three had insertion of the valve into conduits and 7 were deployed into native right ventricular outflow tracts. The pulmonary valve was successfully inserted in all 10 patients with no mortality. Postprocedural complications included paravalvar leak in 2 patients, suspected endocarditis in 1 patient who developed early valve regurgitation and wound infection in 1 patient. CONCLUSIONS: Several approaches to hybrid PVR may be employed in small children with a high success rate. Follow-up studies are required to evaluate longer term durability of these approaches compared to standard surgical replacement.

A Novel Approach to Extensive Chest Wall Reconstruction in a Child.

Reconstruction of extensive chest wall defects is challenging in young children. Rigid prosthetic plates, designed to prevent paradoxic respiration, do not grow with the child and may result in progressive chest and spinal deformity. Also because of the greater proportionate size of the thorax relative to the limbs in young children, extrathoracic soft tissue flaps may be too small for an adequate reconstruction. Here we report reconstruction of a large chest wall defect after resection of a Ewing's sarcoma in a 2-year-old boy using Permacol membrane (Medtronic, Minneapolis, MN) supported by a diagonally translocated seventh rib and covered by a latissimus dorsi flap.

Point-of-Care Ultrasound echocardiography diagnosis of an atrial myxoma in a child presenting with stroke.

We describe a case of paediatric stroke secondary to atrial myxoma, diagnosed in the Emergency Department by Point-of-Care echocardiography. A previously fit and healthy teenage male presented to our paediatric emergency department following a collapse with loss of consciousness. He had suffered a stroke, and had facial paralysis and hemiplegia. His cardiac examination revealed a 3/6 ejection systolic murmur. Whilst his CT was being reported, he had a Point-of-Care echocardiogram in the resuscitation room which showed a very large mass arising from the left atrium and occupying >50% of the chamber. A piece of the myxoma had detached and caused the stroke. The patient was rapidly transferred to a cardiac intensive care unit and underwent emergency surgery the same day. His tumour was successfully completely resected, and he has only a mild residual hemiplegia. Cardiac myxoma should be considered in any child who presents with unexplained acute stroke and a cardiac murmur. Point-of-Care Ultrasound echocardiography in the Paediatric Emergency Department can be used to make a life-saving diagnosis, enabling early surgical management and preventing lifelong complications in children.

High-risk Fontan completion patients achieve low perioperative risk and benefit from cavopulmonary connection 7 years out†.

OBJECTIVES: Our unit has pursued Fontan completion in all patients except those with immobility or combined poor ventricular function and high pulmonary artery pressures. We assessed retrospectively whether conventional high-risk criteria would predict patients with a poorer outcome. METHODS: One hundred and thirty-three consecutive children who underwent extracardiac Fontan completion (2004-2012) had their outcomes recorded (mean follow-up of 7 years). Three groups were analysed: those with 1 of 6 historical risk factors (outside 6 commandments), those with 1 of reduced systemic ventricular function or pulmonary artery pressure >15 mmHg (outside 2 commandments) versus those with no contraindications. The Fischer's exact test examined frequency differences, with the χ2 test to look for outcome associations. RESULTS: There were no differences in postoperative complication rates between the outside 6 commandments (n = 105) or outside 2 commandments (n = 49) versus the low-risk no-contraindication group (n = 28): arrhythmias [18% (P = 0.3) or 18% (P = 0.3) vs 25%], infection [22% (P = 0.6) or 33% (P = 0.2) vs 21%], cerebrovascular accident [6% (P = 0.5) or 10% (P = 0.3) vs 4%], length of stay [20 days (P = 0.4) or 23 days (P = 0.2) vs 21 days] and duration of chest drainage (P = 0.5). There was 1 predischarge mortality in each group. Long term, the majority of patients in each group had suitable haemodynamics for fenestration closure [95% (P = 0.7) or 95% (P = 0.7) vs 92%]. Long term, there was no difference in the rate of arrhythmias [11% (P = 0.5) or 12.5% (P = 0.3) vs 7%], protein-losing enteropathy [1% (P = 0.1) or 2% (P = 0.3) vs 7%] or moderate or more ventricular dysfunction on echocardiography [2% (P = 0.7) or 4% (P = 0.7) vs 4%]. Notably, there was a higher rate of catheter reinterventions in the high-risk groups [22% (P < 0.05) or 24% (P < 0.05) vs 7%]. CONCLUSIONS: The medium-term benefits of Fontan completion can be achieved for high-risk patients, suggesting that historical selection criteria should be re-examined.

Sequential Melody Valve Insertion After Repair of Tetralogy of Fallot Atrioventricular Septal Defect.

Repair of tetralogy of Fallot with atrioventricular septal defect may be associated with hemodynamic challenges in the postoperative period particularly as left atrial hypertension secondary to left atrioventricular valve dysfunction may exacerbate pulmonary regurgitation and augment low cardiac output. We present a case describing hybrid strategies to treat severe left atrioventricular and pulmonary valve regurgitation with modified balloon expandable stent valves to counter low cardiac output secondary to valve dysfunction. Such strategies offer an alternative to standard valve choices and should be considered as an extension of the current surgical valve inventory.

A congenital foregut malformation with a laryngeal bronchus.

We present the case of a 2-month-old boy who required intubation at birth for stridor, at which point a supraglottic cyst was noted. With recurrence of the cyst, a subsequent computed tomographic scan and magnetic resonance imaging demonstrated a mass encroaching on the larynx, descending into the mediastinum, and encasing the major vessels. A median sternotomy and transcervical incision enabled excision of a well-defined mass with a laryngeal attachment. This attachment was consistent with a bronchus on histopathologic investigation. To our knowledge, this is the first reported case of ectopic lung tissue arising from the larynx and descending into the mediastinum.

Do statins have a role in the promotion of postoperative wound healing in cardiac surgical patients?

Cardiac surgical patients often have associated comorbidities that can impede normal wound healing; however, statin therapy has the potential to improve this process through augmentation of the normal inflammatory response. Outcomes included a 30% earlier rate of wound epithelialization and an 80% greater wound-breaking strength combined with faster wound healing rates (13.0 days vs 18.7 days, p<0.0001). Inhibition of farnesyl pyrophosphate may hold a key role in the mediation of such advantageous effects. This systematic review suggests that there is sufficient evidence to warrant completion of a human trial to assess the effects of statins on wound healing.

Oesophageal inflammatory paediatric chylothorax.

Paediatric chylothoraces are rare, particularly outside the operative setting. Cases of spontaneous chylothorax are often demanding diagnostically and frequently associated with patient morbidity. We present a challenging case of paediatric chylothorax associated with inflammatory oesophageal perforation likely related to foreign body ingestion.

Extrinsic bronchial compression due to patent ductus arteriosus closure device.

Interventional cardiology provides a valuable nonoperative approach for the modern management of patent ductus arteriosus (PDA) in patients with non-complex congenital heart disease. We describe a patient with a right-sided aortic arch who developed severe bronchomalacia after PDA device closure that necessitated extensive surgical repair. Consequently, we advise that in infants with a right-sided aortic arch and PDA inserting into the right pulmonary artery, device closure is challenging due to the potential risk of bronchial compression and subsequent development of bronchomalacia. Consideration should be given to surgical closure or use of a softer duct occlusion device.

Pravastatin reduces Marfan aortic dilation.

BACKGROUND: The sequelae of aortic root dilation are the lethal consequences of Marfan syndrome. The root dilation is attributable to an imbalance between deposition of matrix elements and metalloproteinases in the aortic medial layer as a result of excessive transforming growth factor-beta signaling. This study examined the efficacy and mechanism of statins in attenuating aortic root dilation in Marfan syndrome and compared effects to the other main proposed preventative agent, losartan. METHODS AND RESULTS: Marfan mice heterozygous for a mutant allele encoding a cysteine substitution in fibrillin-1 (C1039G) were treated daily from 6 weeks old with pravastatin 0.5 g/L or losartan 0.6 g/L. The end points of aortic root diameter (n=25), aortic thickness, and architecture (n=10), elastin volume (n=5), dp/dtmax (maximal rate of change of pressure) (cardiac catheter; n=20), and ultrastructural analysis with stereology (electron microscopy; n=5) were examined. The aortic root diameters of untreated Marfan mice were significantly increased in comparison to normal mice (0.161 ± 0.001 cm vs 0.252 ± 0.004 cm; P<0.01). Pravastatin (0.22 ± 0.003 cm; P<0.01) and losartan (0.221 ± 0.004 cm; P<0.01) produced a significant reduction in aortic root dilation. Both drugs also preserved elastin volume within the medial layer (pravastatin 0.23 ± 0.02 and losartan 0.29 ± 0.03 vs untreated Marfan 0.19 ± 0.02; P=0.01; normal mice 0.27 ± 0.02). Ultrastructural analysis showed a reduction of rough endoplasmic reticulum in smooth muscle cells with pravastatin (0.022 ± 0.004) and losartan (0.013 ± 0.001) compared to untreated Marfan mice (0.035 ± 0.004; P<0.01). CONCLUSIONS: Statins are similar to losartan in attenuating aortic root dilation in a mouse model of Marfan syndrome. They appear to act through reducing the excessive protein manufacture by vascular smooth muscle cells, which occurs in the Marfan aorta. As a drug that is relatively well-tolerated for long-term use, it may be useful clinically.

Relief of right ventricle to pulmonary artery conduit compression by chest wall resection.

Many congenital heart defects require reconstruction of the right ventricular outflow tract utilizing a right ventricle to pulmonary artery conduit. One of the challenges with these conduits is the development of conduit stenosis. This phenomenon is quite common and typically results from a combination of progressive calcification, fibrosis, and/or the relative size mismatch that occurs with patient growth. However, extrinsic compression is much less common and a much more difficult problem to address. Chest wall resection and reconstruction is an option for alleviating external conduit compression that provides good results.