Clinical presentation, diagnosis and management of multisystem inflammatory syndrome in children (MIS-C): a systematic review.

BACKGROUND: Knowledge about multisystem inflammatory syndrome in children (MIS-C) is evolving, and evidence-based standardised diagnostic and management protocols are lacking. Our review aims to summarise the clinical and diagnostic features, management strategies and outcomes of MIS-C and evaluate the variances in disease parameters and outcomes between high-income countries (HIC) and middle-income countries (MIC). METHODS: We searched four databases from December 2019 to March 2023. Observational studies with a sample size of 10 or more patients were included. Mean and prevalence ratios for various variables were pooled by random effects model using R. A mixed generalised linear model was employed to account for the heterogeneity, and publication bias was assessed via funnel and Doi plots. The primary outcome was pooled mean mortality among patients with MIS-C. Subgroup analysis was conducted based on the income status of the country of study. RESULTS: A total of 120 studies (20 881 cases) were included in the review. The most common clinical presentations were fever (99%; 95% CI 99.6% to 100%), gastrointestinal symptoms (76.7%; 95% CI 73.1% to 79.9%) and dermatological symptoms (63.3%; 95% CI 58.7% to 67.7%). Laboratory investigations suggested raised inflammatory, coagulation and cardiac markers. The most common management strategies were intravenous immunoglobulins (87.5%; 95% CI 82.9% to 91%) and steroids (74.7%; 95% CI 68.7% to 79.9%). Around 53.1% (95% CI 47.3% to 58.9%) required paediatric intensive care unit admissions, and overall mortality was 3.9% (95% CI 2.7% to 5.6%). Patients in MIC were younger, had a higher frequency of respiratory distress and evidence of cardiac dysfunction, with a longer hospital and intensive care unit stay and had a higher mortality rate than patients in HIC. CONCLUSION: MIS-C is a severe multisystem disease with better mortality outcomes in HIC as compared with MIC. The findings emphasise the need for standardised protocols and further research to optimise patient care and address disparities between HIC and MIC. PROSPERO REGISTRATION NUMBER: CRD42020195823.

Enhancing Quality of Congenital Heart Care Within Resource-Limited Settings.

Over 90% of the world's children with congenital heart disease (CHD) are born in the resources poor settings of low- to middle-income countries (LMICs). The shortfall in human and material resources and dysfunctional health systems leads to poor quality of care (QoC) which contributes substantially to suboptimal outcomes of patients with CHD in LMICs. Notwithstanding these challenges, it is possible to develop a quality improvement (QI) framework that can have a significant impact on outcomes and prevent a number of deaths. In this review, we examine the common barriers to implementing effective QI processes in LMICs. Using examples of successful QI initiatives in LMIC, we propose a broad framework that focuses on simple, yet effective measures involving cohesive efforts of all key participants guided and nurtured by a leadership that strongly values QoC.

Recommendations for developing effective and safe paediatric and congenital heart disease services in low-income and middle-income countries: a public health framework.

The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs.

Clinical and epidemiological features of pediatric population hospitalized with COVID-19: a multicenter longitudinal study (March 2020-December 2021) from Pakistan.

Background: We aimed to explore the epidemiological, clinical, and phenotypic parameters of pediatric patients hospitalized with COVID-19 in Pakistan. Methods: This longitudinal cohort study was conducted in five tertiary care hospitals in Pakistan from March 2020 to December 2021. Data on various epidemiological and clinical variables were collected using Case Report Forms (CRFs) adapted from the WHO COVID-19 clinical data platform at baseline and at monthly follow-ups for 3 months. Findings: A total of 1090 children were included. The median age was 5 years (Interquartile range 1-10), and the majority presented due to new signs/symptoms associated with COVID-19 (57.8%; n = 631), the most common being general and respiratory symptoms. Comorbidities were present in 417 (38.3%) children. Acute COVID-19 alone was found in 932 (85.5%) children, 81 (7.4%) had multisystem inflammatory syndrome (MIS-C), 77 (7.0%) had overlapping features of acute COVID-19 and MIS-C, and severe disease was found in 775/1086 (71.4%). Steroids were given to 351 (32.2%) patients while 77 (7.1%) children received intravenous immunoglobulins. Intensive care unit (ICU) care was required in 334 (31.6%) patients, and 203 (18.3%) deaths were reported during the study period. The largest spike in cases and mortality was from July to September 2021 when the Delta variant first emerged. During the first and second follow-ups, 37 and 10 children expired respectively, and medical care after discharge was required in 204 (25.4%), 94 (16.6%), and 70 (13.7%) children respectively during each monthly follow-up. Interpretation: Our study highlights that acute COVID-19 was the major phenotype associated with high severity and mortality in children in Pakistan in contrast to what has been observed globally. Funding: The study was supported by the World Health Organization (WHO), which was involved in the study design but played no role in its analysis, writeup, or publication.

Paediatric posterior reversible encephalopathy syndrome: is there an association of blood pressure with imaging severity and atypical magnetic resonance characteristics?

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is relatively uncommon in paediatric patients; however, its pathophysiology remains obscure. OBJECTIVE: The aims of this study were to find an association or correlation between (1) blood pressures and (2) imaging severity and the presence of atypical imaging features in children with PRES. MATERIALS AND METHODS: We performed a retrospective cross-sectional evaluation in children diagnosed with PRES. We reviewed radiologic findings along with each patient's clinical profile and outcome. We categorised imaging severity into mild, moderate and severe, and assessed the MR imaging pattern, enhancement and diffusion restriction for each child. We assessed both associations and correlations between variables using the chi-square test, Cramer V and Kendall tau b. RESULTS: A total of 63 children met the inclusion criteria (31 boys; mean age 9.7 years). A total of 42 children (67%) had an elevated blood pressure. Imaging showed parieto-occipital lobe involvement pattern in 24 (38%) children, frontal lobe pattern in 25 (40%) and cerebellar involvement in 12 (19%). Three (5%) had haemorrhage, 15 (24%) had contrast enhancement and 19 (30%) had positive diffusion restriction (cytotoxic oedema). We found no statistically significant association between imaging severity and blood pressures (P=0.11), nor any association between blood pressure and atypical imaging findings such as diffusion restriction (P=0.1), enhancement (P=0.11) or haemorrhage (P=0.33). CONCLUSION: According to our results, there is no statistically significant association or correlation between blood pressure and either imaging severity or atypical imaging features in children with PRES. Further prospective studies are warranted.

Quality improvement initiative in paediatric echocardiography laboratory in a low- to middle-income country.

AIMS: To determine the impact of a quality improvement (QI) initiative in the area of paediatric echocardiography (echo) in a low- to middle-income country (LMIC).Care for patients with congenital heart disease is challenging, especially in LMICs. Collaborative learning through QI projects is imperative to ensure improvement in delivery processes leading to better patient outcomes. METHODS AND RESULTS: This QI initiative was taken by a team consisting of physicians and sonographers. Problems were identified, a key driver diagram (KDD) was created, and simple process re-engineering was done using interventions based on the KDD. Metrics (five process and one outcome) were assessed to determine the effectiveness of the QI project. The process metrics assessed were comprehensiveness of exam, timeliness of reporting, diagnostic accuracy and error, and sedation adverse event rates of transthoracic echocardiograms, while a novel comprehensive echo laboratory (lab) quality score was developed as an outcome metric. Data were collected quarterly and analysed in the post-implementation phase. Significant improvement was seen in comprehensive mean score (20.4-29.7), timeliness (40-95%), and diagnostic accuracy rate (91-100%), while a decrease was seen in the diagnostic error rate (7.5-3.5%) and the sedation adverse event rate (6.8-0%), pre- vs. post-implementation. The overall quality outcome score improved from 7 to 19 and the echo lab was able to achieve adequate quality. CONCLUSION: This QI initiative produced improvement in all the processes, and the overall quality of the echo lab without any substantial increase in resources or cost.

Multisystem inflammatory syndrome (MIS-C) in Pakistani children: A description of the phenotypes and comparison with historical cohorts of children with Kawasaki disease and myocarditis.

OBJECTIVES: To determine clinical, laboratory features and outcomes of Multisystem Inflammatory Syndrome in children (MIS-C) and its comparison with historic Kawasaki Disease (KD) and Viral Myocarditis (VM) cohorts. METHODS: All children (1 month- 18 years) who fulfilled the World Health Organization criteria of MIS-C presenting to two tertiary care centers in Karachi from May 2020 till August 31st were included. KD and VM admitted to one of the study centers in the last five years prior to this pandemic, was compared to MIS-C. RESULTS: Thirty children with median age of 24 (interquartile range (IQR)1-192) months met the criteria for MIS-C. Three phenotypes were identified, 12 patients (40%) with KD, ten (33%) VM and eight (26%) had features of TSS. Echocardiography showed coronary involvement in 10 (33%), and moderate to severe Left Ventricular dysfunction in 10 (33%) patients. Steroids and intravenous immunoglobulins (IVIG) were administered to 24 (80%) and 12 (41%) patients respectively while 7 (23%) received both. Overall, 20% children expired. During the last five years, 30 and 47 children were diagnosed with KD and VM, respectively. Their comparison with MIS-C group showed lymphopenia, thrombocytosis, and higher CRP as well as more frequent atypical presentation in MIS-C KD group with less coronary involvement. The MIS-C VM was more likely to present with fulminant myocarditis. CONCLUSIONS: Our MIS-C cohort is younger with higher mortality compared to previous reports. MIS-C is distinct from historic cohorts of KD and VM in both in clinical features and outcomes.

Cardiovascular Dysfunction in Children Exposed to Preeclampsia During Fetal Life.

BACKGROUND: Keeping in view the developmental origin of health and disease hypothesis, the aim of this study was to assess differences in cardiac and vascular structure and function in children exposed to preeclampsia in utero compared with those of normotensive mothers. The hypothesis under investigation was that children exposed to preeclampsia would have altered cardiac and vascular structure and function compared with the unexposed group. METHODS: This was a retrospective cohort study that included children 2 to 10 years of age born to mothers with and without exposure to preeclampsia in utero (n = 80 in each group). Myocardial morphology and function using echocardiography and carotid intima-media thickness and pulse-wave velocity were determined. Multivariate linear regression was used to compare preeclampsia-exposed and nonexposed groups. Subgroup analysis to assess differences between early- and late-onset preeclampsia was also performed. RESULTS: Forty-one percent of mothers (n = 33) had early-onset preeclampsia. Children in the exposed group had a significantly higher prevalence of stage 1 systolic and diastolic hypertension (22% [n = 18] and 35% [n = 18], respectively) compared with the unexposed group (9% [n = 7] and 19% [n = 15], respectively; P = .01). Children in the exposed group also had higher pulse-wave velocity compared with those in the unexposed group (0.42 ± 0.1 vs 0.39 ± 0.1, P = .03). Subgroup analysis revealed that changes in blood pressure and pulse-wave velocity were determined primarily by early-onset preeclampsia. There was no significant difference in cardiac morphology or systolic and diastolic function between the exposed and unexposed groups. CONCLUSION: In utero exposure to preeclampsia has an effect on vascular function in children aged 2 to 10 years, related primarily to early-onset disease. Routine blood pressure screening should be recommended for such children.

Referral indications for foetal echocardiography in a low resource setting.

OBJECTIVE: The aim of this study was to review primary indications for foetal echocardiography among pregnant women at a tertiary care center in a low resource setting. METHODS: A retrospective record review was conducted of all women who have had a foetal echocardiogram between January 2015 and December 2016 at The Aga Khan University Hospital, Karachi, Pakistan. Information regarding maternal clinical characteristics, anomaly scan findings, indications for echocardiography and findings on foetal echocardiogram (FE) was collected. RESULTS: The dataset included 1909 patients. Maternal comorbidities including maternal diabetes, (n=614, 47%) was the most common maternal indication, while multiple pregnancy (n=232, 38%) and intra-cardiac echogenic foci (n=168, 28%) was the most common foetal indication for referral. Major CHD was detected in 4.2% (n=81) of cases. In those with CHD, the most common indication for getting a FE was an abnormal 4-chamber (n=31, 38%) view on the screening obstetric ultrasound. CONCLUSIONS: Most of our patients were referred on the basis of indications that were not warranted based on international guidelines and very few had major CHD on FE. This emphasizes the need for justifying referrals for a resource intensive procedure such as FE.

Outcomes after the Fontan operation in the Middle East: A large Saudi Arabian single centre experience.

BACKGROUND: Fontan outcomes data from large volume Middle Eastern Centres are lacking. We report our experience after the Fontan operation from a tertiary cardiac centre in Saudi Arabia. METHOD: All 458 consecutive patients who had Fontan surgery 1986 through 2015 at the Prince Sultan Cardiac Centre, Riyadh [PSCC], Saudi Arabia, were evaluated for baseline, early and late post-operative outcomes and their uni and multivariate determinants. RESULTS: The mean age at Fontan operation was 7 years [IQR 4.8-9.0]. The most common anatomic diagnoses were tricuspid atresia (104 [23%]) and double-inlet left ventricle (81 [18%]). Only 3 patients in the present series had hypoplastic left heart syndrome [HLHS]. Early mortality [i.e. during Fontan surgical admission] was 3.1%. At late follow-, 35 (8%) patients were lost to follow up. The 1, 5, 10, 20 and 30 year survival was 96%, 94%, 93% and 85%, respectively. In the modern surgical era, 5, 10 and 15 year survival were 96%, 95% and 93% respectively. Univariate determinants of death or transplant were hypoalbuminemia, elevated NtProBNP >500, surgical era prior to 1999, the lack of Fontan fenestration, and prior atriopulmonary Fontan [APF] procedure. On multivariate analysis, surgical era before 1999 and prior APF procedure were independently associated with death or transplant. CONCLUSIONS: Fontan patients from this large volume Middle Eastern centre have comparable early and late mortality outcomes compared to prior published reports. Rigorous selection criteria at the time of Fontan, and Fontan specific dedicated care teams are likely contributors to this success.

Cardiac Abnormalities Seen in Pediatric Patients During the SARS-CoV2 Pandemic: An International Experience.

Background During the SARS-CoV2 pandemic, there has been increase in hyperinflammatory presentation in previously healthy children with a variety of cardiac manifestations. Our objective is to describe the cardiac manifestations found in an international cohort of 55 pediatric cases with multi-system inflammatory syndrome (MIS-C) during the SARS-CoV2 pandemic. Methods and Results We reviewed data on previously healthy pediatric patients (≤18 years) with structurally normal hearts who presented at hospitals in the United States, United Kingdom, Spain and Pakistan with MIS-C and had consultation with a pediatric cardiologist. Data collected included demographics, clinical presentation, laboratory values, electrocardiographic abnormalities, echocardiographic findings and initial therapies. A total of 55 patients presented with MIS-C. Thirty-five patients (64%) had evidence of decreased left ventricular function, 17 (31%) had valvulitis, 12 (22%) with pericardial effusion and 11 (20%) with coronary abnormalities. Twenty-seven (49%) required ICU admission and 24 (44%) had evidence of shock. Eleven patients (20%) fulfilled complete Kawasaki disease criteria and had lower NT pro-BNP, D-dimer and ferritin levels compared with those who did not fulfill criteria. Electrophysiologic abnormalities occurred in 6 patients and included complete atrioventricular (AV) block, transient AV block and ventricular tachycardia. Conclusions We describe the first international cohort of pediatric patients with MIS-C during the SARS-CoV2 pandemic with a range of cardiac manifestations. This paper brings awareness and alertness to the global medical community to recognize these children during the pandemic and understand the need for early cardiology evaluation and follow-up.

Surgical management of ascending aortic pseudoaneurysm in a 2-year-old boy: a case report.

BACKGROUND: Aortic pseudoaneurysms are rare but life-threatening complications usually seen after cardiac surgery. The causes could be multifactorial such as infection or trauma. CASE PRESENTATION: We report the surgical management of a postoperative pseudoaneurysm of the ascending aorta caused by methicillin-resistant Staphylococcus aureus in a 2-year-old Middle Eastern boy who had undergone ventricular septal defect closure, subaortic membrane resection, and pulmonary artery de-banding. He was immediately operated on for resection of the aneurysm. A computed tomography scan at 2 months following surgery showed no aneurysm. Antibiotics were continued for 6 weeks and our patient was discharged with negative blood cultures. CONCLUSION: Early diagnosis and appropriate treatment of such rare complication can be lifesaving.

Four successful pregnancies in a patient with Fontan palliation and congenital heart disease: a case report.

BACKGROUND: Fontan is a palliative procedure in patients with single ventricle. Single ventricle supports systemic cardiac output and pulmonary blood flow is passively directed to the right pulmonary artery. Women with Fontan palliation are reported to have increased maternal risk during pregnancy. There are few reports of successful pregnancies in such cases. However data on these pregnancies is lacking, we consider this to be the first reported from kingdom of Saudi Arabia. CASE PRESENTATION: We present a 35-year-old woman from the Kingdom of Saudi Arabia who had Fontan surgery and who had four successful pregnancies and multiple miscarriages. She delivered live, low birth weight neonates. CONCLUSION: This report provides an anecdotal evidence that pregnancy can be tolerated in an adequate Fontan patient with successful outcomes.

Outcome of infants with unrepaired heart disease admitted to the pediatric intensive care unit: single-center developing country perspective.

OBJECTIVE: Congenital heart disease (CHD) has an incidence of ∼0.8-1%. Outcome of previously diagnosed CHD patients awaiting surgery (either correction or palliation) in a developing country setting is unknown. We strive to determine the outcome of patients with CHD awaiting surgery who present to pediatric intensive care unit (PICU) setting with an acute illness. DESIGN: Retrospective cross-sectional chart review. SETTING: Pediatric intensive care unit of The Aga Khan University Hospital, Karachi, Pakistan. PATIENT: Medical records of infants (1-12 months) with CHD awaiting surgery presenting to the PICU with an acute illness between January 2009 and June 2012 were included. Newly diagnosed CHD patients, those not requiring PICU admission, and those transferred to another hospital were excluded. RESULTS: A total of 34 infants met the inclusion criteria. Median age at presentation was 5 months. Seventy-four percent of the infants had CHD lesion characterized by increased pulmonary blood flow (shunt lesions). Though none of the patients met the strict criteria for sepsis or pneumonia, 74% were admitted with a diagnosis of pneumonia or sepsis. Only 15% of patient had congestive heart failure as an admitting diagnosis. Oxygen therapy was given to 94% of these patients. Fifty-nine percent of these patients expired during the admission, 95% of those expired had multiorgan dysfunction. CONCLUSION: Patients with CHD awaiting surgery and who admitted to the PICU with acute illness are at high risk for mortality. Stringent criteria to diagnose pneumonia or sepsis should be used in these patients.

Anthracycline-induced cardiotoxicity: prospective cohort study from Pakistan.

OBJECTIVES: To identify anthracycline-induced acute (within 1 month) and early-onset chronic progressive (within 1 year) cardiotoxicity in children younger than 16 years of age with childhood malignancies at a tertiary care centre of Pakistan. DESIGN: Prospective cohort study. SETTING: Aga Khan University, Karachi, Pakistan. PARTICIPANTS: 110 children (aged 1 month-16 years). INTERVENTION: Anthracycline (doxorubicin and/or daunorubicin). OUTCOME MEASUREMENTS: All children who received anthracycline as chemotherapy and three echocardiographic evaluations (baseline, 1 month and 1 year) between July 2010 and June 2012 were prospectively analysed for cardiac dysfunction. Statistical analysis including systolic and diastolic functions at baseline, 1 month and 1 year was carried out by repeated measures analysis of variance. RESULTS: Mean age was 74±44 months and 75 (68.2%) were males. Acute lymphoblastic leukaemia was seen in 70 (64%) patients. Doxorubicin alone was used in 59 (54%) and combination therapy was used in 35 (32%). A cumulative dose of anthracycline <300 mg/m(2) was used in 95 (86%). Fifteen (14%) children developed cardiac dysfunction within a month and 28 (25%) children within a year. Of these 10/15 (66.6%) and 12/28 (43%) had isolated diastolic dysfunction, respectively, while 5/15 (33.3%) and 16/28 (57%) had combined systolic and diastolic dysfunction. Seven (6.4%) patients expired due to severe cardiac dysfunction. Eight of 59 (13.5%) children showed dose-related cardiotoxicity (p=<0.001). Cardiotoxicity was also high when the combination of doxorubicin and daunorubicin was used (p=0.004). CONCLUSIONS: Incidence of anthracycline-induced cardiotoxicity is high. Long-term follow-up is essential to diagnose its late manifestations.

Midterm results of bovine jugular vein conduit for right ventricular outflow tract reconstruction.

OBJECTIVE: To evaluate the midterm results of Contegra conduit. METHODS: The retrospective study comprised patient record at Aga Khan University Hospital, Karachi, of conduits implanted between May 2007 and June 2012. Data collection was made from the clinical notes and from serial echocardiograms by a single cardiologist. The last followup echocardiography was done at the time of data collection in June 2012. SPSS 19 was used for statistical analysis. RESULTS: A total of 18 conduits had been implanted (16-22 mm) during the study period. Median age at the time of surgery was 9 years (range: 2.5-16 years). Early mortality was seen in 3 (16.66%) patients, but none was Contegra related. Of the remaining 15 patients, 2 (13.33%) with a diagnosis of Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries (PA), developed severe distal pressure gradient (50 mmHg) across Contegra over a median period of 18 months (range: 12-24 months), with resultant severe regurgitation and needed percutaneous intervention. There was no thrombosis, calcification, anuerysmal dilation or late deaths. CONCLUSION: At midterm followup, Contegra conduit was associated with low re-intervention rates with satisfactory haemodynamic results. However, long-term durability must be determined for this conduit, especially in patients with Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries.

Contegra valved conduit in the paediatric population: an exciting prospect for right ventricle to pulmonary artery reconstruction; experience and outcomes at Aga Khan University.

OBJECTIVE: The focus of this study is to share the experience and outcomes of Contegra graft implantation in the paediatric and adult population in Pakistan. METHODS: Between May 2007 and July 2011, 16 patients, underwent implantation of a Contegra valved conduit. All operations were performed through a median sternotomy with cardiopulmonary bypass. Indications included: Pulmonary atresia with ventricular septal defect (n = 11), Tetralogy of Fallot with absent Pulmonary Valve (PV) syndrome (n = 2), double outlet right ventricle, transposition of great arteries and pulmonary stenosis (n = 1), isolated aortic valve disease (n = 1) and a pseudo-aneurysm with infective endocarditis (n = 1).Conduit sizes varied between 16-22 mm. RESULTS: The three in hospital deaths were unrelated to the Contegra valved conduit. One patient was lost to follow up. Of the 12 survivors, 10 are currently free from re-operation or complications related to the conduit while one needed distal pulmonary artery dilatation owing to critical stenosis and another had severe Valvular regurgitation. Echocardiographic evaluation of the Contegra valved conduit demonstrated no haemodynamically significant valve regurgitation in 10 patients. CONCLUSION: In this small review of 16 operations using the Contegra valved conduit for Right Venticular Outflow Tract (RVOT) reconstruction in the paediatric population, we observed good post operative results concerning conduit function. The Contegra conduit provides an excellent substitute to the homograft with satisfactory early and mid-term results though long-term results are awaited in Pakistan.

Murmur and Doppler alternans in critical pulmonary stenosis.

Pulsus alternans is a well-recognized clinical entity in which alternating strong and weak pulses are detected. It usually is secondary to underlying myocardial failure. Murmur alternans (alternation in murmur intensity) has been described in aortic stenosis and a few right-sided lesions such as pulmonary hypertension and embolism. This report describes a case of murmur alternans in critical pulmonary stenosis that also showed Doppler alternans on echocardiography. The underlying cause was right ventricular systolic dysfunction.