Kawasaki disease is easily diagnosed when it presents in its complete form, but because not all characteristic symptoms are always present at the same time, and the diagnosis of incomplete and atypical Kawasaki disease is often challenging, a delay in diagnosis or misdiagnosis often occurs. We present the diagnostic approach to Kawasaki disease with common pitfalls and explain how to avoid them. We also describe current practice and new trends in treatment.
The waiting time in infants for a cardiac transplant remains high, due to the scarcity of donors. Consequently, waiting list morbidity and mortality are higher than those in other age groups. Therefore, the decision to list a small infant for cardiac transplantation is seen as an ethical dilemma by most physicians. This review aims to describe outcomes, limitations, and ethical considerations in infant heart transplantation. We used Medline and Embase as data sources. We searched for publications on infant (< 1 year) heart transplantation, bridge-to-transplant and long-term outcomes, and waiting list characteristics from January 2009 to March 2021. Outcome after cardiac transplant in infants is better than that in older children (1-year survival 88%), and complications are less frequent (25% CAV, 10% PTLD). The bridge-to-transplant period in infants is associated with increased mortality (32%) and decreased transplantation rate (43%). This is mainly due to MCS complications or the limited MCS options (with 51% mortality in infancy). Outcomes are worse for infants with CHD or in need of ECMO-support.Conclusion: Infants listed for cardiac transplantation have a high morbidity and mortality, especially in the period between diagnosis and transplantation. For those who receive cardiac transplant, the outlook is encouraging. Unfortunately, despite growing experience in VAD, mortality in children < 10 kg and children with CHD remains high. After transplantation, patients carry a psychological burden and there is a probability of re-transplantation later in life, with decreased outcomes compared to primary transplantation. These considerations are seen as an important ethical dilemma in many centers, when considering cardiac transplantation in infants (< 1 year).What is Known:• For infants, waitlist mortality remains high. In the pediatric population, MCS reduces the waiting list mortality.What is New:• Outcomes after infant cardiac transplantation are better than other age groups; however, MCS options remain limited, with persistently high waiting list mortality. • Future developments in MCS and alternative options to reduce waiting list mortality such as ABO-incompatible transplantation and pulmonary artery banding are encouraging and will improve ethical decision-making when an infant is in need of a cardiac transplant.
1. Purpose Reflex anoxic seizures are an overreaction of the vagal system, resulting in bradycardia or brief cardiac arrest. Because of self-limiting character of the spells with no complications on short or long term, treatment is only necessary in case of frequent spells or severe clinical presentation. Treatment options are medication such as atropine or the implantation of a cardiac pacemaker, with the latter being invasive and entailing risk for important complications. We investigated atropine treatment and aimed to examine if pacemaker placement can be avoided. 2. Methods We retrospectively reviewed patients treated in our center for severe reflex anoxic seizures with atropine sulphate from January 2017 until May 2023, and compared our results to those in the literature. 3. Results In our population, 100% of the patients reported adequate symptom management after atropine treatment, with complete resolution in 10%. Minor side effects were reported in 70%, with need for change of treatment in one patient (10%). Severe complications were not reported, unlike up to 40% of permanent or severe complications after pacemaker placement. 4. Discussion Atropine is a safe and efficient treatment to manage the symptoms, with similar success rate to pacemaker implantation. However, pacemaker implantation entails a substantial risk for complications. This might be considered redundant for a benign and temporary condition, when other efficient treatment options are possible.
Case description: A 4-year old boy without significant prior history, presented with pronounced dyspnea. During clinical examination only mild hepatomegaly was present. Thoracic radiography showed significant cardiomegaly. Echocardiography revealed left ventricular dilatation and poor systolic function. Both Troponin and NT-pro-BNP level were elevated. Treatment with diuretics and inotropics was started with clinical improvement. The diagnosis of an anomalous left circumflex artery out of the right pulmonary artery was suspected by coronarography and confirmed by coronary CT. Coronary reimplantation was performed without complications. Subsequently, left ventricular dimensions normalized progressively in the next months. Discussion: Without congenital heart disease, an anomalous left circumflex artery arising from the pulmonary artery is rarely present. Symptoms and prognosis are determined by the amount of collateral circulation to provide adequate myocardial perfusion. Diagnosis of an anomalous left circumflex artery (LCx) is mostly made at adult age. However, our patient presented at young age. Even though the origin of both coronary arteries was normal, a coronary anomaly lies at the origin of his cardiac dysfunction. Treatment consisting of surgical reimplantation or coronary bypass is the primary choice. Conclusion: We present this case to highlight the importance of having a high index of suspicion of coronary artery abnormalities in children with dilated cardiomyopathy, as the prognosis can be very favorable after surgical repair. Transthoracic echocardiography is not always conclusive in showing a normal origin of both coronary arteries and certainly not for visualizing the LCx reliably. Therefore, further imaging should be considered in children with a new diagnosis of unexplained DCM.
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