-
JACC. Advances Jan 2024Hypertensive disorders of pregnancy (HDP) can be classified into gestational hypertension, preeclampsia (PRE), and chronic hypertension with superimposed preeclampsia...
BACKGROUND
Hypertensive disorders of pregnancy (HDP) can be classified into gestational hypertension, preeclampsia (PRE), and chronic hypertension with superimposed preeclampsia (SPE).
OBJECTIVES
The purpose of this study was to retrospectively examine the echocardiographic differences in biventricular structure and function in 3 HDP groups of women in comparison to normotensive pregnant controls.
METHODS
Women with an echocardiogram during or within the first year of pregnancy were identified within our integrated health network. Exclusion criteria included age <18 years, diagnosis of pulmonary embolism, malignancy, autoimmune disease, and structural heart disease.
RESULTS
We identified a total of 706 subjects (cases: n = 427, normotensive controls: n = 279). Cases were divided into 3 groups: gestational hypertension (n = 57), PRE (n = 291), and SPE (n = 79). In adjusted analyses, echocardiographic parameters demonstrated a graded difference in left ventricular (LV) mass index, relative wall thickness, mitral inflow E, mitral inflow A, septal e', lateral e', E/e', left atrial volume index, tricuspid velocity, and lateral e' velocities with the most profound findings noted in the SPE group. Specifically, adjusted LV mass index (adjusted β = 14.45, 95% CI: 9.00-19.90) and E/e' (adjusted β = 2.97, 95% CI: 2.27-3.68) was highest in the SPE group in comparison to controls ( < 0.001).
CONCLUSIONS
LV remodeling and diastolic filling abnormalities are more common in HDP and are most evident in SPE and PRE. Echocardiography during or immediately after pregnancy may be useful in these high-risk women to identify these abnormalities. The long-term implications of these echocardiographic abnormalities require further study.
PubMed: 38939814
DOI: 10.1016/j.jacadv.2023.100725 -
JACC. Advances Jan 2024Multilevel obstruction in left ventricular inflow and outflow predisposes to arrhythmias in Shone's complex (SC).
BACKGROUND
Multilevel obstruction in left ventricular inflow and outflow predisposes to arrhythmias in Shone's complex (SC).
OBJECTIVES
The purpose of this study was to study the prevalence and outcomes (heart failure [HF] hospitalization, cardiac transplant, death) of cardiac arrhythmias in adults with SC.
METHODS
Adults with SC (defined as ≥2 lesions out of supramitral ring, parachute mitral valve, subvalvular/valvular aortic stenosis (AS), and aortic coarctation) seen at Mayo Clinic between January 1999 and March 2020 were identified and evaluated for the presence of sustained atrial fibrillation, atrial flutter, and ventricular arrhythmias (VA). Kaplan-Meier survival analysis was used to calculate the occurrence of these arrhythmias.
RESULTS
Seventy-three patients with SC (mean age at first visit 33 ± 13 years) were identified. Most common anomalies were valvular AS (88%), coarctation (85%), parachute mitral valve (44%), subvalvular AS (44%), and supramitral ring (25%). Atrial arrhythmias were diagnosed in 24 patients (33%) at a mean age of 34.6 ± 12.7 years. Patients with atrial fibrillation and atrial flutter had higher number of surgeries, left atrial size, right ventricular systolic pressure, and HF hospitalizations. A rhythm control approach was used in majority of patients (75% on antiarrhythmic drugs and 50% underwent catheter ablation). Sustained VA occurred in 6 of 73 patients of whom 4 had an ejection fraction <40%. Death and cardiac transplantation occurred in 11 and 3 patients, respectively, during a median follow-up of 7.3 ± 6.0 years.
CONCLUSIONS
In adults with SC, atrial arrhythmias occurred in one-third of patients, were associated with more HF hospitalizations, and frequently required rhythm control. Prevalence of sustained VA was 8% and implantable cardioverter-defibrillator implantation should be considered in those with reduced ejection fraction.
PubMed: 38939811
DOI: 10.1016/j.jacadv.2023.100715 -
Heart Rhythm Jun 2024In patients with narrow QRS complex, both ventricular and biventricular pacing is associated with increased cardiac morbidity and mortality. This risk is not decreased...
BACKGROUND
In patients with narrow QRS complex, both ventricular and biventricular pacing is associated with increased cardiac morbidity and mortality. This risk is not decreased by ventricular pacing avoidance algorithms which cause non-physiologic atrioventricular delays.
OBJECTIVE
To report outcomes in patients with narrow QRS complex, when paced complex is in normal range and physiologic atrioventricular delays are programmed.
METHODS
In 196 patients with QRS duration of 92 ± 10 msec, permanent pacing was done at site of His bundle electrogram. The pacemakers were then programmed to maintain physiologic AV delays and increase heart rates in response to exercise. Patients received usual care and were followed for 3-years.
RESULTS
The paced complex exhibited a delta wave and the ventricular activation time, QRS axis and lead-I voltage remained in normal range. Physiologic programming resulted in His bundle pacing burden of 92%. In patients with decreased ejection fraction, there was significant improvement in left ventricular function, left ventricular dilatation and mitral regurgitation (p <0.003). In patients with normal ejection fraction, left ventricular function remained normal without new valvular abnormalities. The 3-year all-cause mortality was 10%, and there was no increase in heart failure admissions.
CONCLUSION
In patients with narrow QRS complex, when paced QRS morphology is maintained in normal range and atrio-ventricular dyssynchrony is avoided, His bundle pacing is associated with a low all-cause mortality and improvement in abnormal echocardiographic parameters. The paced QRS morphology and physiologic atrioventricular delays may be important factors to evaluate in future trials of conduction system pacing.
PubMed: 38908462
DOI: 10.1016/j.hrthm.2024.06.027 -
Advances in Experimental Medicine and... 2024Hypoplastic left heart syndrome (HLHS) is a severe congenital cardiovascular malformation characterized by hypoplasia of the left ventricle, aorta, and other structures...
Hypoplastic left heart syndrome (HLHS) is a severe congenital cardiovascular malformation characterized by hypoplasia of the left ventricle, aorta, and other structures on the left side of the heart. The pathologic definition includes atresia or stenosis of both the aortic and mitral valves. Despite considerable progress in clinical and surgical management of HLHS, mortality and morbidity remain concerns. One barrier to progress in HLHS management is poor understanding of its cause. Several lines of evidence point to genetic origins of HLHS. First, some HLHS cases have been associated with cytogenetic abnormalities (e.g., Turner syndrome). Second, studies of family clustering of HLHS and related cardiovascular malformations have determined HLHS is heritable. Third, genomic regions that encode genes influencing the inheritance of HLHS have been identified. Taken together, these diverse studies provide strong evidence for genetic origins of HLHS and related cardiac phenotypes. However, using simple Mendelian inheritance models, identification of single genetic variants that "cause" HLHS has remained elusive, and in most cases, the genetic cause remains unknown. These results suggest that HLHS inheritance is complex rather than simple. The implication of this conclusion is that researchers must move beyond the expectation that a single disease-causing variant can be found. Utilization of complex models to analyze high-throughput genetic data requires careful consideration of study design.
Topics: Humans; Genetic Predisposition to Disease; Hypoplastic Left Heart Syndrome; Phenotype
PubMed: 38884762
DOI: 10.1007/978-3-031-44087-8_60 -
Advances in Experimental Medicine and... 2024Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect characterized by several abnormalities that result in a significantly underdeveloped left...
Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect characterized by several abnormalities that result in a significantly underdeveloped left ventricle and severe hypoplasia of the ascending aorta, often leading to retrograde perfusion. These abnormalities include aortic valve atresia or severe stenosis, accompanied by a severely hypoplastic aortic valve annulus (Fig. 59.1). Mitral valve atresia, hypoplasia, and/or stenosis with a hypoplastic valve annulus with or without a ventricular septal defect can also contribute to the development of HLHS. Endocardial fibroelastosis and sinusoids may be present as well. The interatrial septum can either be closed or the foramen ovale severely stenotic. Other malformations, such as anomalous pulmonary venous drainage or variations of the systemic veins, may coexist. It is also common to observe a coarctation of the aorta in these cases.
Topics: Humans; Infant, Newborn; Hypoplastic Left Heart Syndrome
PubMed: 38884761
DOI: 10.1007/978-3-031-44087-8_59 -
BMJ Case Reports Jun 2024Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation....
Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation. After birth, he developed severe respiratory failure, congestive heart failure and airway obstruction because an enlarged left atrium from severe mitral regurgitation compressed the distal left main bronchus. There is limited experience in surgical management of this condition in Thailand, and the patient's mitral valve was too small for replacement. Therefore, he was treated with medication to control heart failure and supported with positive pressure ventilation to promote growth. We have followed the patient until the current time of writing this report at the age of 2 years, and his outcome is favourable regarding heart failure symptoms, airway obstruction, growth and development. This case describes a challenging experience in the non-surgical management of MA with severe regurgitation, which presented at birth.
Topics: Humans; Mitral Valve Insufficiency; Hydrops Fetalis; Male; Infant, Newborn; Mitral Valve; Echocardiography; Heart Failure; Heart Defects, Congenital; Positive-Pressure Respiration
PubMed: 38866580
DOI: 10.1136/bcr-2023-259272 -
Circulation. Cardiovascular Imaging Jun 2024Prominent multi-scallop systolic leaflet displacement toward the left atrium (atrialization) is typically observed in bileaflet mitral valve prolapse (MVP) with mitral...
BACKGROUND
Prominent multi-scallop systolic leaflet displacement toward the left atrium (atrialization) is typically observed in bileaflet mitral valve prolapse (MVP) with mitral annular disjunction. We hypothesized that mitral leaflet atrialization is associated with an underlying left atrial (LA) myopathy characterized by progressive structural and functional abnormalities, irrespective of mitral regurgitation (MR) severity.
METHODS
We identified 334 consecutive patients with MVP, no prior atrial fibrillation, and comprehensive clinical and echocardiographic data. LA function was assessed by LA reservoir strain, LA function index, and LA emptying fraction. We also classified the stage of LA remodeling based on LA enlargement and LA reservoir strain (stage 1: no remodeling; stage 2: mild remodeling; stage 3: moderate remodeling; and stage 4: severe remodeling). The primary end point was the composite risk of sudden arrhythmic death, heart failure hospitalization, or the new onset of atrial fibrillation.
RESULTS
Bileaflet MVP with no or mild MR had a lower LA reservoir strain (=0.04) and LA function index (<0.001) compared with other MVP subtypes. In multivariable linear regression adjusted for cardiovascular risk factors and MR ≥moderate, bileaflet MVP remained significantly associated with lower LA function parameters (all <0.05). There was a significant increase in the risk of events as the LA reservoir strain and LA remodeling stage increased (<0.001). In multivariable analysis, stage 4 of LA remodeling remained significantly associated with a higher risk of events compared with stage 1 (hazard ratio, 6.09 [95% CI, 1.69-21.9]; =0.006).
CONCLUSIONS
In a large MVP registry, bileaflet involvement is associated with reduced LA function regardless of MR severity, suggesting a primary atriopathy in this MVP subtype. Abnormal LA function, particularly when assessed through a multiparametric approach, is linked to a higher risk of cardiovascular events and may improve risk stratification in MVP, even in those without significant MR.
Topics: Humans; Mitral Valve Prolapse; Female; Male; Aged; Middle Aged; Atrial Function, Left; Atrial Remodeling; Heart Atria; Atrial Fibrillation; Risk Factors; Severity of Illness Index; Retrospective Studies; Mitral Valve; Echocardiography; Mitral Valve Insufficiency; Predictive Value of Tests
PubMed: 38860362
DOI: 10.1161/CIRCIMAGING.123.016319 -
The Annals of Thoracic Surgery Jun 2024Left ventricular outflow tract (LVOT) obstruction in obstructive hypertrophic cardiomyopathy (HCM) is caused by a constellation of abnormalities. This study reviewed...
BACKGROUND
Left ventricular outflow tract (LVOT) obstruction in obstructive hypertrophic cardiomyopathy (HCM) is caused by a constellation of abnormalities. This study reviewed outcomes of a comprehensive approach to correct these abnormalities during surgery.
METHODS
This was a single-institution study of patients with HCM who underwent septal myectomy from 2016 to 2023. Their New York Heart Association functional classification and most recent echocardiogram that estimated LVOT gradient and mitral valve function were tracked.
RESULTS
The study included 103 patients with a mean age of 54 years (interquartile range, 40-67 years) and common comorbidities: hypertension (50%) and atrial fibrillation (25%). On average, the preprocedure resting echocardiogram showed an LVOT gradient of 36.4 mm Hg and moderate or severe mitral regurgitation in 50.5% of patients. All patients underwent septal myectomy, and associated abnormalities contributing to LVOT obstruction were addressed. Elongation of the anterior leaflet of the mitral valve was typically treated with papillary muscle realignment (72%). Aberrant papillary muscle heads and elongated secondary chordae tendineae contributing to systolic anterior motion were resected (66%). Myocardial bands, including apicoseptal bands contributing to LVOT obstruction, were resected (68%). With an average follow-up of 4 years, 91% of patients were considered to be in New York Heart Association functional class I or II. Long-term echocardiographic follow-up showed a mean peak LVOT gradient of 11 mm Hg (interquartile range, 4-13 mm Hg). Only 1 patient had more than mild mitral regurgitation.
CONCLUSIONS
A comprehensive surgical approach to HCM that addresses the entire constellation of abnormalities associated with HCM, including mitral valve anterior leaflet elongation, aberrant or displaced mitral valve subvalvular apparatus, and myocardial bands, leads to outstanding midterm outcomes.
PubMed: 38851415
DOI: 10.1016/j.athoracsur.2024.05.024 -
European Journal of Case Reports in... 2024Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include...
BACKGROUND
Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome. .
CASE 1
A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms.
CASE 2
A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13-14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management.
CONCLUSION
PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients.
LEARNING POINTS
Alagille syndrome (ALGS) is a complex multisystem disorder involving the liver, heart, skeleton, face, and eyes. Cardiovascular involvement occurs in up to 95% of the patients.Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF).A parachute mitral valve (PMV) presenting as mitral stenosis and mid-aortic syndrome is not commonly described anomalies in association with ALGS. Here, we present such rare cases.
PubMed: 38846669
DOI: 10.12890/2024_004545