Myhre syndrome is a rare disease secondary to pathogenic variants in SMAD4 gene. It is a multisystem disease characterised by short stature, deafness, joint stiffness, craniofacial dysmorphism, and potential cardiac manifestations. Herein, we report two new paediatric cases of Myhre syndrome who, additionally, presented with mid-aortic syndrome. This confirms and extends the scarce reports describing the association between these two entities.

Kaposiform hemangioendothelioma is a rare tumour of vascular origin that rarely occurs in the heart. We provided a rare case of a 26-day-old infant with tachypnoea. Echocardiography showed a solid tumour in the pericardial cavity and a large amount of pericardial effusion. The solid tumour was confirmed by surgery, and the pathology was kaposiform hemangioendothelioma. We analysed this case and reviewed the related literature to explore the clinical features and echocardiographic manifestations to improve the understanding, diagnosis, and treatment of this disease for clinicians and sonographers.

Patent foramen ovale closure is recommended for patients who are at risk for recurrent paradoxical embolism and cryptogenic stroke. The standard technique of patent foramen ovale closure is established from the femoral vein. However, alternative methods may be necessary for patent foramen ovale closure as in every interventional procedure. A 45-year-old female patient with an intramural giant uterine myoma had a history of recurrent deep vein thrombosis and stroke. A diffuse thrombus was detected in both iliac veins associated with inferior vena cava compression of the myoma. Also, a patent foramen ovale was revealed on echocardiography as a cause of embolic events. Hysterectomy was initially planned by gynaecology, but due to the possible risk of embolisation of inferior vena cava thrombus and stroke after removal of the compressive mass during hysterectomy, initial patent foramen ovale closure and then hysterectomy was scheduled in the Gynecology-Cardiology-Cardivascular Surgery council. Patent foramen ovale closure was performed via the right jugular vein approach. But because of the tight left atrial ostium of the patent foramen ovale, the catheter could not pass to the left atrium from the right atrium. With an anchor of a 5.0 × 15 mm coronary balloon over a 0.014-inch guidewire to the pulmonary vein, we were able to reach the left atrium. The patent foramen ovale was closed successfully, and the patient underwent a hysterectomy after closure without any embolic event. The patient was asymptomatic at 6 months of control.

Cardiac tumours are extremely rare. Most of the cases are diagnosed post-mortem.

In this case, a tumour was found in a neonate during routine ultrasound screening in the first trimester of pregnancy. After birth, resection of the formation was performed and histologically confirmed as a cavernous haemangioma. Additionally, propranolol was prescribed in order to prevent relapse.

A 4-year-old boy presented to our institution with pancytopenia, consumptive coagulopathy, hepatosplenomegaly and recurrent complex pericardial effusion secondary to kaposiform lymphagiomatosis. Due to extensive loculation, conventional drainage was minimally effective. As an adjunct to medical therapy, the Indigo™ aspiration system was used to remove thrombus within the pericardial space. Our patient had good medium-term results with complete resolution of his pericardial effusion at 4 months.

Prolonged pleural effusion is a fairly common condition which has considerable impact on complicated and longer hospital stays after Fontan surgery. Identifying the patient population prone to have pleural effusions is still seeking for an answer. This study is to determine the variables that may predict prolonged pleural effusion according to the data of 69 patients who underwent Fontan operation between June 2018 and December 2020 and survived to date. Prolonged pleural effusion was defined as the need for a chest tube for more than 7 days. Two patient groups, with and without prolonged effusion, were compared in terms of pre-, peri-, and post-operative variables. The patients were subdivided into "high-risk" and "low-risk" groups based on the pre-operative catheterisation data. The most frequent main diagnosis was tricuspid atresia (n: 13, 19%). Among 69 patients, 28 (40%) had prolonged pleural effusion whereas 11 (16%) had effusions that lasted longer than 14 days. Ten patients among prolonged effusion group (35%) had pulmonary atresia coexistent with the main diagnosis. Fontan operation was performed in 6 patients (8.7%) over the age of 10, and 4 of these patients (67%) had prolonged pleural effusion. Among numerous variables, statistical significance between the two groups was achieved in pre-operative mean pulmonary artery pressure, post-operative albumin, C-reactive protein levels, length of hospital stay, duration of chest tube drainage, and amount of effusion per day. Early recognition and treatment strategies with routine medical protocol use remain to be the cornerstone for the management of post-operative prolonged pleural effusions after Fontan surgery.

Re-expansion pulmonary edema is a serious complication that can occur after minimally invasive cardiac surgery through a right mini-thoracotomy. Herein, we describe two paediatric cases where re-expansion pulmonary edema was observed after simple atrial septal defect closure through a right mini-thoracotomy. This is the first case report of re-expansion pulmonary edema after a paediatric cardiac surgery.

We describe a rare case of acute pulmonary artery thromboembolism in a 17-year-old male patient who presented to our emergency department following a syncopal episode. A chest radiograph showed a convex pulmonic cone and an increased cardiothoracic index, and two-dimensional echocardiogram suggested near-occlusion of both pulmonary arterial branches. Multi-slice pulmonary angio-tomography revealed massive thrombosis of the pulmonary artery. He was treated with systemic anticoagulation and subsequently required surgical thrombectomy, with favourable early outcome. Although the cause of the thromboembolism remains unproven, we discuss possible etiologies.

We present two exceptional cases of 14-year-old girls diagnosed with rare cardiomyopathies (left ventricular non-compaction, and arrhythmogenic right ventricular cardiomyopathy), both presenting with the unusual finding of bidirectional ventricular tachycardia.

Right ventricular failure after placement of left ventricular assist device in paediatric heart failure is associated with increased mortality. We report successful use of intravenous prostacyclin for right ventricular support and pulmonary hypertension after initiation of left ventricular assist device support. This suggests that intravenous prostacyclins may be an important therapy in right ventricular failure following ventricular assist device implantation.

Coronary artery fistulas are rare, but one of the most common forms of congenital coronary abnormalities. These patients are often diagnosed incidentally undergoing coronary angiography, but with the advent of novel cardiac imaging tools, there is an increasing rate of detection as well as transcatheter management of these fistulas. Our case is unusual in a way that it involved a combination of two separate coronary artery fistulas arising from both the coronary systems draining into the same site.

Takotsubo cardiomyopathy has an incidence of 1% of acute coronary syndrome in the adult population, and the risk of recurrence is approximately 1.5% per year. However, only a few cases have been reported in children. Having a neurologic disorder and being exposed to the same trigger repeatedly have been associated with an increased risk.

We report two cases of successful emergency pacing via the umbilical vein in neonates with congenital complete atrioventricular block. The first patient, a neonate with normal cardiac anatomy, underwent emergency temporary pacing via the umbilical vein under echocardiographic guidance. The patient underwent permanent pacemaker implantation on postnatal day 4. The second patient, a neonate with heterotaxy syndrome, underwent emergency temporary pacing through the umbilical vein under fluoroscopic guidance. The patient underwent permanent pacemaker implantation on postnatal day 17.

A 14-year-old previously healthy female presented with chest pain and dyspnoea for 2 days in the setting of a recent upper respiratory infection. She had elevated inflammatory markers and troponin, resulting in the diagnosis of acute myocarditis. Transthoracic echocardiography demonstrated mild systolic dysfunction and a moderate pericardial effusion. Additionally, her echocardiogram showed concentric left ventricular hypertrophy raising concern for hypertrophic cardiomyopathy. She was treated with intravenous immunoglobulin. Serial echocardiograms revealed rapid resolution of her ventricular hypertrophy. Cardiac magnetic resonance confirmed the diagnosis of myocarditis.

Aortopulmonary window is a condition characterized by a communication between the pulmonary artery and the ascending aorta. The coexistence of aortopulmonary window and an anomalous right coronary artery originating from the pulmonary artery is rarely observed together, as mentioned in previous studies. In this report, we aim to describe our diagnostic and treatment experiences with a 6-year-old patient diagnosed with aortopulmonary window associated with an abnormal origin of the right coronary artery from the pulmonary artery.

We performed coronary artery bypass grafting with the internal thoracic artery in three infants. Grafts were patent in all cases. One patient was lost due to chronic heart failure. Coronary artery bypass grafting can be performed even in infancy, and early surgical intervention may be necessary when myocardial ischaemia is recognised.

Ornithine transcarbamylase deficiency is an X-linked disorder which results in the accumulation of ammonia causing irritability and vomiting. Acute hyperammonemia requires rapid and intensive intervention. However, as those clinical features are non-specific and commonly seen in peri-operative situation, ornithine transcarbamylase deficiency could be difficult to diagnose prior to and post-emergency cardiac surgery. We report a 2-day-old male neonate who was diagnosed with ornithine transcarbamylase deficiency presenting hyperammonemia and severe heart failure after total anomalous pulmonary venous connection repair.