Unilateral absence of the pulmonary artery is a rare congenital cardiovascular anomaly that can lead to pulmonary hypertension and poor outcomes. We report the case of a 1-month-old infant with isolated unilateral absence of the pulmonary artery and severe pulmonary hypertension on the right and left sides, respectively. The patient was unresponsive to multiple medications for pulmonary hypertension, and surgical revascularisation was unfeasible. However, iloprost inhalation was effective.

A 50-year-old woman who had atrial septal defect surgery at 11 months old underwent ascending aortic aneurysm resection and two attempts at closure of a residual atrial septal defect. Post-operatively, she had severe cyanosis. She was referred to our centre where a transesophageal echocardiogram and cardiac catheterisation showed an iatrogenic interatrial tunnel-type communication that was closed with an Amplatzer Vascular Plug.

Partial anomalous venous connection with sinus venosus atrial septal defect is repaired with different approaches including the Warden procedure. Complications include stenosis of the superior caval vein and pulmonary venous baffle; however, cyanosis is rarely seen post-operatively. We report a patient presenting with cyanosis 5 years after a Warden, which was treated with a transcatheter approach.

Right-to-left shunt in atrial septal defect without pulmonary hypertension is a rare condition and can present with complications such as cyanosis. This is a rare case of cyanosis caused by right-to-left shunt atrial septal defect related to prominent crista terminalis

A 4-hour-old infant with profound cyanosis on an alprostadil infusion was urgently transferred to Rady Children’s Hospital with suspected CHD. Upon arrival, urgent echocardiography was performed but could not confirm the presence of discrete pulmonary veins or pulmonary venous drainage. Given the difficulty in delineating the anatomy, a cardiac CT scan was performed and demonstrated a nearly atretic common pulmonary vein with multiple small collaterals that drained to systemic veins. Due to the high risk of mortality associated with operative repair, the decision was made to proceed with compassionate withdrawal of care. The described anatomy of common pulmonary vein atresia remains rare, and to our knowledge, fewer than 40 cases have been reported in the literature. Albeit rare, common pulmonary vein atresia should be considered in the differential diagnosis of a severely cyanotic neonate.

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.

Most cases of severe or critical pulmonary stenosis are detected early and interventional management is routine within the first days of life. We present a case of a thirteen-year-old boy diagnosed with pulmonary stenosis and atrial septal defect with low ventricle ejection fraction. The patient underwent staged pulmonary balloon valvuloplasty and interventional atrial septal defect closure with good results.

Isolated atrial defects usually lead to left-to-right shunt and right ventricular volume load. Descriptions of cyanosis with this congenital heart defect are rare.

We describe a rare case of inferior caval vein flow directed through an atrial septal defect in the fossa ovalis leading to severe cyanosis, but without any additional intracardiac anatomical abnormalities. The baby with clinical features of Marfan’s syndrome had an eventration of the right-sided diaphragm. Surgical closure of the defect resolved the cyanosis, but the child died 10 weeks later of severe valvar dysfunction, related to Marfan’s syndrome.

Mechanisms of cyanosis in patients with atrial septal defects are discussed. Echocardiographic bubble studies both from the lower and upper half of the body may help to clarify the mechanism of an otherwise unexplained cyanosis.

Congenital right pulmonary artery to left atrium fistula is rare cause of cyanosis. It is an abnormal fistulous connection between right pulmonary artery and left atrium representing a direct communication between a pulmonary artery and vein with absence of capillary network connecting between these two. Cardiovascular examination usually remains normal. High index of suspicion on clinical examination and subsequent transthoracic echocardiography is needed to suspect this entity. Bubble contrast echocardiography usually confirms the diagnosis. Cardiac catheterization is used as diagnostic as well as therapeutic modality. Cardiac catheterization is useful in classifying the fistula and helps in transcatheter closure by embolization devices. Choice of devices depends on type of fistula, vascular access, and presence of atrial communication. Here, we are reporting 8-month-old girl presenting with type 2 right pulmonary artery to left atrium fistula, which underwent successful transcatheter closure by 6 mm/4 mm duct occluder (Heart R, Lifetech Scientific, Shenzhen, China). Early closure in this young age will prevent complications of cyanosis. The technical consideration, possible access, and closure techniques are discussed in this young infant.

Biatrial drainage of the right superior caval vein is an extremely rare cardiac anomaly that generally presents in childhood. We present a case of anomalous connection of the right superior caval vein with superior sinus venosus atrial septal defect and partial anomalous pulmonary venous return in a 5-month-old male presenting with unexplained cyanosis and hypoxia.

Aneurysms of the pulmonary arteries and trunk are rare entities. The Waterston shunt is a palliative procedure for children with cyanotic CHD due to obstruction of the pulmonary outflow. Described complications are distortion of the pulmonary artery and pulmonary arterial hypertension. We report a patient with a giant right pulmonary artery aneurysm in relation to a Waterston shunt.

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.

A 5-year-old girl presented with chronic fatigue and extreme exercise intolerance. After countless doctor visits, investigations, and hospital admissions, striking desaturation during exercise test pointed to a cardiovascular problem. Desaturation as a result of right-to-left shunting through a patent foramen ovale during upright exercise was hypothesised. A cardiac catheterisation confirmed the presence of an unusually cranially located patent foramen ovale; the defect was subsequently closed. Physical re-evaluation 6 weeks later showed spectacular physical and mental improvement and stabile saturation during exercise.

In recent years, there has been a rise in the number patients with CHD surviving into adulthood. Many have complications related to their CHD or its treatments, outside the heart, including ocular abnormalities. The objective of this review is to highlight the ocular abnormalities that occur in adults with CHD, either from their condition or related to the common drugs prescribed to manage it. In particular, we reviewed the effects of cyanosis, coarctation of the aorta, endocarditis, and the side effects of Sildenafil and Amiodarone. A change in the retinal vasculature is a common observation with cyanosis or coarctation of the aorta. Occlusion of the retinal vessels may also be observed in cyanotic patients, as well as those with infectious endocarditis. Sildenafil has established ocular side effects; here they are explored in the context of therapy for pulmonary hypertension. Similarly, Amiodarone has established ocular risks, which are summarised. The high prevalence of ocular consequences in adult CHD patients reinforces the need for knowledge of the risks involved and for frequent ophthalmological screening where appropriate.

Communications between the pulmonary artery and left atrium cause cyanosis. The images document serial haemodynamic changes in such a fistula from fetal life to the postnatal period with a successful transcatheter intervention.

The formation of a fistula between the right pulmonary artery and the left atrium via a sac is a very rare cyanotic congenital cardiopulmonary defect. A fistula between the pulmonary artery and left atrium may cause cardiac failure in utero. It can safely be treated surgically and in selected cases closure can be performed with transcatheter insertion of a device. In this article, we present a case with a fistula between the right pulmonary artery and the left atrium that was considered unsuitable for transcatheter closure and was safely treated surgically.

In many parts of the world, mostly low- and middle-income countries, timely diagnosis and repair of congenital heart diseases (CHDs) is not feasible for a variety of reasons. In these regions, economic growth has enabled the development of cardiac units that manage patients with CHD presenting later than would be ideal, often after the window for early stabilisation – transposition of the great arteries, coarctation of the aorta – or for lower-risk surgery in infancy – left-to-right shunts or cyanotic conditions. As a result, patients may have suffered organ dysfunction, manifest signs of pulmonary vascular disease, or the sequelae of profound cyanosis and polycythaemia. Late presentation poses unique clinical and ethical challenges in decision making regarding operability or surgical candidacy, surgical strategy, and perioperative intensive care management.