We describe the case of a newborn girl who displayed association of aortic atresia and interrupted aortic arch, with retrograde flow in ascending aorta, through extracranial anastomoses between vertebral arteries (arisen from descending aorta) and external carotids.

Coronary compression exclusion during right ventricle outflow tract stenting is recommended and potential oversight may be fatal. Balloon inflation in right ventricle outflow tract with simultaneous aortography can be inconclusive or falsely negative. We present a case of 27-year-old male post Ross operation qualified for percutaneous pulmonary valve implantation. Neither of the conventional views obtained provided a definite exclusion of coronary compression, therefore an intravascular ultrasound of the left coronary artery before and during balloon inflation in right ventricle outflow tract was performed. Acquired images allowed excluding potential constriction, thus a covered stent and pulmonary valve were implanted and the procedure was concluded. Two hours later, the patient complained of chest pain. Transthoracic echocardiography demonstrated a significant pericardial effusion. Retrospective analysis of the final angiogram revealed a possibility of subtle extravasation at the distal part of the homograft. A hybrid procedure consisting of additional covered stent implantation, pericardial drainage, and second pulmonary valve implantation was performed with an acceptable result. To conclude, in case of doubtful or unconvincing images obtained from aortography or selective angiography during balloon inflation in right ventricle outflow tract, intravascular ultrasound might be a feasible and useful technique. Signs of homograft rupture may be subtle, whereas symptoms of cardiac tamponade delayed. In selected patients percutaneous treatment of homograft rupture is achievable and beneficial.

In this article, we report a rare case of double aortic arch. The case presented initially with a foreign object in the oesophagus. The patient was a 2-year-old boy, who was referred with primary symptoms of tussis (15 days) and emesis (2 days). He had a history of ingesting a coin. Routine chest X-ray indicated a rounded, metal foreign object in the upper oesophagus. A half-Yuan coin was removed by gastroduodenoscopy. Echocardiographic imaging suggested that the patient had double aortic arch, which was subsequently diagnosed by CT angiography with three-dimensional reconstruction. The right subclavian artery arose from the right loop of the double aortic arch. The left subclavian artery as well as left and right common carotid arteries had distinct origins from the left aortic arch. Imaging also indicated atresia of the distal left arch. The patient underwent corrective surgery and made a full recovery. Despite the rarity, double aortic arch should be considered when patients present with a foreign object in the oesophagus. Echocardiography and CT angiography can inform the diagnosis.

Cyanosis in a newborn is commonly due to intracardiac shunts or duct-dependent lesions. Systemic venous anomalies are uncommon and sinus venous atrial septal defects do not usually present with cyanosis. We report the case of a 4-month-old female with persistent hypoxemia due to a right superior vena cava overriding a superior sinus venous atrial septal defect resulting in a right to left shunt.

To demonstrate the use of transthoracic contrast echocardiography in the detection of pulmonary arteriovenous fistulas in patients with a previously constructed anastomosis between the superior caval vein and the right pulmonary artery (Glenn shunt), and to examine their prevalence in this special population, we evaluated prospectively all patients followed up in the Grown-Up Congenital Heart Unit subsequent to construction of a classical or bi-directional Glenn shunt. We studied 12 patients, aged from 21 to 38 (mean 28 ± 4.8) years who had had a previous cavopulmonary shunt in place for a period of 4 to 33 years (mean 24±9 years). All were examined with cross-sectional contrast echocardiography, 11 patients had cardiac catheterisation and angiography, and 6 patients had magnetic resonance imaging. Systemic arterial oxygen saturations at rest, and during exercise using the modified Bruce protocol, were measured in all patients. Contrast echocardiography showed evidence of pulmonary arteriovenous fistulas in 7 of the 12 patients, with appearance of echo contrast in the left atrium 1–8 seconds after peripheral venous injection in the arm. Simultaneous appearance of microbubbles in the right atrium revealed a residual communication between the superior caval vein and the right atrium in 2 patients, and presence of collaterals between the superior and inferior caval veins in one. Cardiac catheterisation and angiography showed obvious fistulas in 4 patients, and revealed suggestive findings in 2. In patients deemed to have pulmonary arteriovenous fistulas on contrast echocardiography, arterial oxygen saturations at rest (51–94%, mean 75±15.3%) and on exercise (23–91%, mean 53±24.2%) were significantly lower compared to patients judged to be without fistulas (p<0.005). Pulmonary hypertension in the contralateral lung was more common in patients with fistulas (mean left pulmonary arterial pressure 22–110 mm Hg, p=0.014). In patients with cavopulmonary anastomoses, pulmonary arteriovenous fistulas occur frequently in the long term (10–33, mean 25.7±8 years), and are associated with worsening systemic arterial desaturation. Contrast echocardiography should be included in the regular evaluation of these patients as a simple and sensitive technique for the detection of pulmonary arteriovenous fistulas, particularly with the devel opment of increasing cyanosis.

Occlusion using coils is now the treatment of choice for closure of the patent arterial duct. The DuctOcclud (pfm AG, Cologne, Germany) device is a relatively new retrievable coil for such trans-catheter closure. This study expands on previously reported experience with this device, summarizes the advantages of the device, and compares trans-pulmonary and trans-aortic delivery in 47 patients. There were 27 females. The mean, and median, ages were 4.6, and 2.85 years, respectively. The youngest patient was aged 9 months, weighing 7 kg. A trans-aortic delivery was used in 41 cases, and a trans-pulmonary approach in 6 cases. Of the 47 procedures, 45 (96%) were successful at the first attempt. The other two patients were treated successfully at the second attempt, giving a 100% rate of success. The technical characteristics of the coil allowed for its repeated maneuvering until an optimal position was obtained prior to release. Closure was confirmed by lack of ductal flow by echo-doppler on follow- up echocardiography. No short- or long-term complications of the procedure were noted in any of the patients. We conclude that the DuctOcclud device is an effective and safe method for closure of the small-tomoderately patent arterial duct. In a large proportion of patients, trans-aortic delivery is the preferred approach.

This is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein’s anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis “bidirectional Glenn”, was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.

The NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome is a rare novel autoinflammatory disorder. Cardiac involvement has not been previously reported. We present a 12-year-old girl with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome who was diagnosed with severely impaired left ventricular function and complete left bundle branch block during an exacerbation of the disease. Cardiac dysfunction proved to be rapidly reversible after initiation of high-dose methylprednisolone.

Cardiac involvement with multisystem inflammatory syndrome in children can include coronary artery abnormalities, ventricular dysfunction, conduction abnormalities, arrhythmias, pericarditis, and myocarditis. We report the cardiac findings in 34 patients with multisystem inflammatory syndrome in children admitted to a single institution. We looked at patient age, sex, brain natriuretic peptide levels, troponin levels, ejection fraction, presence of pericardial effusion, valvular changes, need for inotropic agents, and electrocardiogram findings. Our data showed that elevated brain natriuretic peptide did not predict troponin elevation and vice versa. Additionally, troponin rise was not a reliable marker for decreased left ventricular ejection fraction. All changes tracked were proven to be transient and resolved after initiating steroids, Intravenous immune globulin (IVIG), and occasionally anakinra.

Accessory mitral valve tissue is a rare congenital cardiac anomaly that is typically discovered incidentally during echocardiographic evaluation prompted by an asymptomatic murmur. This pathology has characteristic echocardiographic elements and is usually associated with other CHD. The decision to perform surgical resection depends on factors such as the degree of obstruction, presence of symptoms, presence of other CHDs, and risk of thrombosis. The researchers hereby present a case of an asymptomatic paediatric patient with accessory mitral valve tissue that produced left ventricular outflow tract obstruction.

We report a 25-year-old woman who was diagnosed with atrial septal defect (ASD). An ECG showed only first-degree atrioventricular block and incomplete right bundle branch block. One day after the percutaneous ASD closure, she had a slight fever and an ECG showed a type 1 Brugada pattern. ECG characteristics of ASD are similar to those of a Brugada ECG. This case is rare combination of Brugada syndrome with ASD.

Anomalous origin of the coronary arteries is a well-known phenomenon, but is less well reported in world literature. We present our findings in a one year old boy in whom there was bifid, or “y”, origin of the right coronary artery, co-existing with a fistula from the right coronary artery which opened into the right ventricle.

Pulmonary atresia with intact ventricular septum and congenital left ventricular aneurysms are both rare, representing <1 and <0.1% of CHD, respectively. The association of left ventricular aneurysms and pulmonary atresia with intact ventricular septum has been documented in the literature, but all the previous case reports have involved children with acquired left ventricular aneurysms. We report an interesting case of a child with both pulmonary atresia with intact ventricular septum and a congenital left ventricular aneurysm. The combination of these two lesions ultimately affected the course of clinical care for the patient.