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Quadricuspid aortic valve is a remarkably rare congenital cardiac anomaly that predominantly becomes regurgitant with the passage of time. Aortic valve stenosis and aortic root dilatation are less common in quadricuspid aortic valve as compared to bicuspid aortic valve. The clinical presentation depends upon the functional status of the aortic valve, left ventricular function, and associated cardiac or coronary anomalies. The quadricuspid aortic valve is easily visualised during transthoracic echocardiogram with a characteristic X pattern of aortic valve in diastole. The association of quadricuspid aortic valve with patent ductus arteriosus is exceedingly rare. We are reporting a case of young girl with mildly regurgitant quadricuspid aortic valve, large patent ductus arteriosus, and volume-loaded left heart who underwent a successful device closure of her patent ductus arteriosus. To the best of our knowledge, such a case is being reported from Pakistan for the first time.
We report on a 50-year-old female patient, with several severe comorbidities and high-surgical risk, in whom we successfully performed a simultaneous transcatheter pulmonary and tricuspid valve-in-ring implantation to treat both bioprosthetic pulmonary valve dysfunction and native torrential tricuspid valve regurgitation, the latter previously managed with a Carpentier annuloplasty ring.
No established risk prediction tool exists in United Kingdom and Irish Paediatric Cardiology practice for patients undergoing cardiac catheterisation. The Catheterisation RISk score for Paediatrics is used primarily in North American practice to assess risk prior to cardiac catheterisation. Validating the utility and transferability of such a tool in practice provides the opportunity to employ an already established risk assessment tool in everyday practice.
Aims:
To ascertain whether the Catheterisation RISk score for Paediatrics assessment tool can accurately predict complications within United Kingdom and Irish congenital catheterisation practice.
Methods:
Clinical and procedural data including National Institute for Cardiovascular Outcomes Research derived outcome data from 1500 patients across five large congenital cardiology centres in the United Kingdom and Ireland were retrospectively collected. Catheterisation RISk score for Paediatrics were then calculated for each case and compared with the observed procedural outcomes. Chi-square analysis was used to determine the relationship between observed and predicted events.
Results:
Ninety-eight (6.6%) patients in this study experienced a significant complication as qualified by National Institute for Cardiovascular Outcomes Research classification. 4% experienced a moderate complication, 2.3% experienced a major complication and 0.3% experienced a catastrophic complication resulting in death. Calculated Catheterisation RISk score for Paediatrics scores correlated well with all observed adverse events for paediatric patients across all CRISP categories. The association was also transferable to adult congenital heart disease patients in lower Catheterisation RISk score for Paediatrics categories (CRISP 1–3).
Conclusion:
The Catheterisation RISk score for Paediatrics score accurately predicts significant complications in congenital catheterisation practice in the United Kingdom and Ireland. Our data validated the Catheterisation RISk score for Paediatrics assessment tool in five congenital centres using National Institute for Cardiovascular Outcomes Research-derived outcome data.
Stenting of the arterial duct (PDA) has become a standard palliation for ductal-dependent pulmonary circulation. Carotid arterial access provides a direct route for stenting vertical ducts. We evaluated our early results of hybrid ductal stenting via surgical carotid cutdown.
Methods and results:
In this retrospective single centre cohort study, hybrid PDA stenting was attempted in 11 patients with “flip technique”, between January 2020 and February 2021, and was successful in 10. Median age was 29 days (interquartile range 17.5–87) and mean weight 3.37 ± 1.23 kg. Mean fluoroscopy time was 13.58 ± 5.35 minutes, mean procedure time was 48.50 ± 22.5 minutes, and mean radiation dose was 1719.5 ± 1217.6 mGycm2. Mean time for cutdown was 9.9 ± 2.4 minutes and for haemostasis and suturing was 25.3 ± 11.0 minutes. Median duration of ventilation post-stenting was 26 hours (interquartile range 21–43.75). The median ICU stay post-procedure was 5 days (interquartile range 4–7.25) and mean hospital stay was 12 ± 6.3 days. On early follow-up, carotid patency was confirmed in all patients with colour Doppler, with no intravascular thrombi, narrowing, haematomas, or aneurysms noted. There were no complications secondary to vascular access. There was one early mortality, 27 days post-stenting, which was unrelated to the procedure.
Conclusion:
This study adds to the limited literature on ductal stenting with carotid access and the flip technique. In our early experience, the hybrid carotid approach is an attractive alternative to percutaneous carotid puncture and has simplified a complex and challenging intervention, with good outcomes.
Ostium secundum atrial septal defects are mostly closed in the cardiac catheterisation laboratories using either Amplatzer® (Abbott Laboratories, IL) atrial septal occluder, Gore® Cardioform septal occluder and more recently using the recently approved (US FDA approval June 2019) Gore® Cardioform atrial septal defect occluder (W. L. Gore & Associates, AZ). Similar to any new device in the market, there is a learning curve to the deployment of this device. We therefore aim to report the key features about this new Gore Cardioform atrial septal defect occluder device with special emphasis on technical aspects that can be employed during transcatheter closure of challenging ostium secundum atrial septal defects using this device.
We report on a 7-month-old male with transient phrenic nerve palsy induced by diagnostic cardiac catheterisation. The phrenic nerve palsy, which is a rare complication, was due to extravascular bleeding from a branch of the internal mammary artery.
We report a 5-month-old infant who developed an unexpected acute ischaemia of the right lower limb following a surgical perimembranous interventricular communication closure. This rare case of ischaemia was due to an occlusive right common iliac artery dissection. It was early managed by angioplasty with two ABSORB® bioresorbable stents, resulted in complete revascularisation of the right leg.
Ductal dissection is rarely mentioned but intractable complication of ductal stenting. We demonstrate the dissection image under fluoroscopy and the use of a relatively large stent as an applicable treatment.
Acute stent thrombosis may complicate neonatal arterial duct stenting for reduced pulmonary blood flow. Thrombolytic agents recanalise the clot but may cause bleeding around the vascular sheaths and other sites. Since early thrombus is platelet mediated, intravenous platelet glycoprotein inhibitor like eptifibatide is likely to be effective, but rarely utilised in neonates. Ductal stent thrombosis treated with eptifibatide is reported.
Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients.
Methods:
All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified. Six early deaths were excluded resulting in a group of 345 patients. Median (interquartile range,) length of follow-up was 14.4 (7.1–19.7) years. Freedom from the composite endpoint of death, total cavopulmonary connection take-down or indication for a heart transplant was analysed.
Results:
Fenestration was absent in 237 patients (68.7%, Group 1), was created and closed later in 79 patients (22.9%, Group 2), and remained open in 29 patients (8.4%, Group 3). Mean survival probability until composite endpoint was 97.1 and 92.9% at 10 and 20 years, respectively. Patients with patent fenestration had worse survival (p < 0.001) as compared to both the non-fenestrated and fenestration closure groups. Despite a similar outcome, exercise capacity was lower in Group 2 than 1 (p = 0.013). In 58 patients with interventional fenestration closure, Nakata index was lower at the time of closure than pre-operatively, and both the pressure in the circuit and oxygen saturation in the aorta increased significantly (p < 0.001).
Conclusions:
Patients with persisting risk factors preventing fenestration closure are at higher risk of reaching the composite endpoint. Patients after fenestration closure have the worse functional outcome; their survival is, however, not different from the non-fenestrated group.
Percutaneous coronary intervention for stenosis of coronary artery after Kawasaki disease presents various challenges. The diameters of reference vessels and femoral artery in children are smaller, and the morphological changes are different from adults. Herein, we describe our successful experience with a severe coronary artery stenosis at the proximal portion of left anterior descending treated with drug-coated balloon dilation.
Kawasaki disease is usually self-limited, but it can lead to aneurysm, stenosis, thrombosis, and myocardial infarction in the coronary arteries. The most important complication of Kawasaki disease is coronary artery aneurysm. Coronary artery aneurysm or ectasia may be seen in 15–25% of patients who do not receive treatment. It develops in 5% of children who receive intravenous immunoglobulin at the appropriate time. Acute myocardial infarction is the most important cause of morbidity and mortality in Kawasaki patients with giant aneurysms. We present a 10-year-old girl who had a history of giant aneurysm in the coronary arteries and underwent percutaneous coronary intervention due to anterior myocardial infarction.
Many interventionists are infatuated by the recent resurgence of the coilwire design with the Super Arrow-Flex® sheath (Teleflex, Inc., NC, United States of America). This exclusive sheath is a highly flexible, durable, conduit intended for use in diagnostic and interventional procedures with several advantages and maximum effectiveness in challenging cases. We report failure to easily advance memory shape occluders through Super Arrow-Flex® sheaths larger than the recommended implant French size. We detail the technical reasons behind this non-previously reported drawback and describe benchside tests as possible solutions.
Pseudoaneurysm of the ascending aorta is rare (1–2%) and a potentially fatal complication following cardiac surgeries. Surgical repair is still the gold standard treatment of ascending aortic pseudoaneurysm. However, endovascular repair methods including stent grafts and Septal Occluder devices have been reported. We report a case of 38-year-old female patient who presented with giant ascending aortic pseudoaneurysm, and aortopulmonary fistula 22 years after modified BlalockTaussig shunt was managed by the transcatheter method. Septal Occluder device 20 mm diameter was delivered to seal the ostium.
Balloon dilatation of coarctation of aorta is a standard of care for the patients presenting with severe left ventricular dysfunction. It can be performed through femoral, carotid, and axillary arterial access. Very few case series were available in the literature through axillary arterial access, despite being its advantage as non-end artery and easily palpable in coarctation of aorta. We present our experience with five cases of neonatal coarctation of aorta with severe left ventricular dysfunction where successful balloon dilatation of coarctation of aorta was performed via axillary approach without adverse events.
Neonatal aortic thrombus is a rare and critical condition that can present mimicking severe coarctation of the aorta or interrupted aortic arch. Transcatheter thrombectomy for this lesion has not been well described. We report a premature neonate with an occlusive proximal descending aorta thrombus, who underwent transcatheter mechanical thrombectomy using an Amplatzer Piccolo PDA occluder (Abbott, North Chicago, IL, USA). The procedure was successful with no subsequent distal thromboembolic events.
Coronary artery fistulas have been described in patients after heart transplantation more often than in the normal population. We reviewed our centre’s database between 2008 and 2020. Thirty patients had coronary angiography and 13 showed non-cameral coronary artery fistulas in their first coronary angiography. Distribution, degree and evolution of the fistulas and characteristics of transplant procedure, patient and immunosuppressive treatments were analysed.
Here, we report two rare cases of pulmonary sequestration that were fed by large systemic arteries and embolized with a large Amplatzer duct occluder and their 3-year follow-up, and we discuss the efficacy and safety of the embolization of a large aberrant systemic artery to pulmonary sequestration using an Amplatzer duct occluder. A 9-year-old boy complained of chest pain for 1 month, and a 6-year-old boy initially complained of recurrent cough for 3 months. A series of examinations was launched to evaluate any possible malformation or abnormalities in the patients. Chest CT and CTA identified a right lower pulmonary sequestration with infection. After admission, transcatheter device occlusion was planned after essential antibiotic treatment, and postoperative infection prevention and anti-inflammatory treatment were given. In the following 2 years of follow-up, neither of the children had recurrent chest pain, cough or other related symptoms. However, the CT follow-up demonstrated that a residual mass was visible in both patients. The same chest scan section revealed slight reductions in lung lesions from 38.344 cm2 to 37.119 cm2 (3% reduction) and 14.243 cm2 to 13.178 cm2 (7.5% reduction) for each patient. No follow-up data demonstrated the long-term clinical outcomes of the residual lesion. We do not recommend that embolization be performed for large pulmonary sequestration lesions with an aberrant artery larger than 6 mm that is planned to receive a device larger than 10 mm, as their outcomes showed a higher possibility of rebuilding the vascularization network feeding the pulmonary sequestration, indicating a higher risk for long-term complications.
We describe a 3-month-old infant with transposition of great arteries and restrictive foramen ovale who presented with severe cyanosis. Child had a large thrombus causing near-total occlusion of the inferior caval vein. An emergency atrial septoplasty was performed via internal jugular venous access. The case emphasises the technical challenges faced while performing this procedure through jugular approach and plausible solutions to overcome these challenges.
Until now, the application of virtual reality as a distraction model has been widely described in the medical field, showing different benefits offered on patient’s perception, particularly related to pain and anxiety. Previous clinical experience of virtual reality applications on surgical intervention has shown how during procedures with local anaesthesia, this modality improves patients’ experience without changing times, costs, and clinical outcomes. Herein, we report our experience with three patients during diagnostic cardiac catheterisation, showing the effect of this technology on patients’ perception and metrics during the procedure.