Left Ventricular Hypoplasia Research Articles

Impact of left ventricular rehabilitation on surgical outcomes in patients with borderline left heart hypoplasia

ObjectiveClinical significance of left ventricular (LV) rehabilitation for borderline left ventricular (LV) hypoplasia is controversial. This study aimed to review the surgical results of patients with borderline LV hypoplasia, and to evaluate the impact of the LV rehabilitation on outcomes. MethodsPatients diagnosed with borderline LV hypoplasia and surgically treated from 2018 to 2022 were included. Overall surgical outcomes were evaluated and the changes in LV volumes calculated using angiography and zlog-NT-proBNP levels were analyzed in patients who underwent LV rehabilitation. ResultsThirty-three patients were included. Sixteen patients underwent primary biventricular repair, three patients underwent primary single ventricle palliation, and the remaining 14 patients underwent LV rehabilitation, including nine bilateral pulmonary artery banding (PAB) and ductal stenting, four central PAB, and one ductal stenting. Among fourteen patients who received LV rehabilitation, one died, one underwent single ventricle palliation, one was waiting for further procedure, and 11 underwent biventricular repair. After biventricular repair, 2 patients died, and 1 patient developed hemodynamic failure. As a result, only 8 patients were alive and in good condition. In patients who underwent LV rehabilitation, LV end-diastolic/end-systolic volume index, and LV stroke volume index increased over time after LV rehabilitation (p=0.001, p=0.007, and p=0.009, respectively). The zlog-NT-proBNP levels were stable until biventricular repair, but significantly higher in patients who presented with hemodynamic failure following biventricular repair compared to patients who did not exhibit hemodynamic failure. ConclusionsIn patients with borderline left heart hypoplasia, LV rehabilitation procedure promoted an increase in LV volume and contributed to establishing a biventricular circulation. The short-term results of this strategy are satisfactory, but further studies are essential to determine the long-term outcomes.

Comprehensive surgical technique for the neonatal and infant Ross Konno procedure – technical pearls

The Ross Konno procedure is a technically demanding operation in neonates and infants, particularly in small babies and those with significant aortic annular and left ventricular outflow tract hypoplasia. There are several key technical considerations for harvesting the pulmonary autograft with care to preserve the left main coronary artery and the septal perforating arteries in addition to ensuring an optimal muscle cuff on the base of the autograft. Accommodation of the autograft deep within the native aortic valve annulus by performing an appropriate Konno incision and ensuring correct suture placement is essential. Adequate epicardial mobilisation and subsequent reimplantation of the coronary arteries into the autograft neo-aortic root is also a key consideration given the significant radial displacement of the coronary arteries required to accommodate the often much larger autograft into the space of a previously very small aortic root. Although the overall technical details of the Ross Konno procedure have previously been described by others the specific granular technical detail and meticulous approach required for a successful procedural outcome in neonates and infants remains to be fully elucidated. Key technical considerations such as suture spacing, positioning of the autograft with in the Konno incision and management of the coronary arteries require detailed description. We describe our institutional approach to the Ross Konno procedure in neonates and infants to clarify these important technical considerations.

Abstract We029: Common and Divergent Cellular Etiologies Underlying Hypoplastic Left Heart Syndrome and Hypoplastic Right Heart Syndrome

Single ventricle heart defects are the most severe forms of congenital heart defects and are classified based on the affected ventricles: hypoplastic left heart syndrome (HLHS) and hypoplastic right heart syndrome (HRHS). Cellular etiologies underlying differential left and right ventricular hypoplasia are unknown and remain intractable without an experimental model to probe common and divergent cellular etiologies between HLHS and HRHS. In this study, we leveraged patient-specific induced pluripotent stem cells (iPSCs) and single-cell transcriptomics to interrogate distinct etiologies of HLHS and HRHS. Both HLHS and HRHS iPSC-derived cardiomyocytes (iPSC-CMs) exhibit a reduced proliferation capacity compared to sex-matched family controls under both static and cyclic stretch conditions. Biological pathways related to cell cycle progression, DNA replication, and cell proliferation are downregulated in both HLHS and HRHS iPSC-CMs, suggesting an intrinsic cardiac proliferation deficiency. Single-cell transcriptomics indicate that differentiation of cardiac mesoderm towards second heart field (SHF) progenitors are compromised in both HLHS and HRHS. However, differentiation of epicardial progenitors is enhanced at the expense of SHF progenitors in HLHS, whereas first heart field (FHF) progenitors are prevalent in HRHS. Trajectory inference analysis uncovers distinct cell lineage determination pathways in FHF and SHF progenitors between HLHS and HRHS. Moreover, HLHS iPSC-CMs exhibit reduced mitochondrial activities whereas HRHS iPSC-CMs show enhanced mitochondrial respiration and ATP production compared to controls. In sum, these common and divergent cellular etiologies of HLHS and HRHS may underlie ventricular hypoplasia in the left side and right side of the heart.

Ventilator strategies in congenital diaphragmatic hernia

This review focuses on contemporary mechanical ventilator practices used in the initial management of neonates born with congenital diaphragmatic hernia (CDH). Both conventional and non-conventional ventilation modes in CDH are reviewed. Special emphasis is placed on the rationale for gentle ventilation and the current evidence-based clinical practice guidelines that are recommended for supporting these fragile infants. The interplay between CDH lung hypoplasia and other key cardiopulmonary elements of the disease, namely a reduced pulmonary vascular bed, abnormal pulmonary vascular remodeling, and left ventricular hypoplasia, are discussed. Finally, we provide insights into future avenues for mechanical ventilator research in CDH.

Long-term outcomes following the Ross procedure in neonates and infants: A multi-institutional analysis

ObjectivesFor neonates and infants with aortic valve pathology, the Ross procedure historically has been associated with high rates of morbidity and mortality. Data regarding long-term durability are lacking. MethodsThe international, multi-institutional Ross Collaborative included 6 tertiary care centers. Infants who underwent a Ross operation between 1996 and 2016 (allowing a minimum 5 years of follow-up) were retrospectively identified. Serial echocardiograms were examined to study evolution in neoaortic size and function. ResultsPrimary diagnoses for the 133 patients (n = 30 neonates) included isolated aortic stenosis (14%, n = 19), Shone complex (14%, n = 19), and aortic stenosis plus other (excluding Shone complex; n = 95, 71%), including arch obstruction (n = 55), left ventricular hypoplasia (n = 9), and mitral disease (moderate or greater stenosis or regurgitation, n = 31). At the time of the Ross procedure, median age was 96 days (interquartile range, 36-186), and median weight was 4.4 kg (3.6-6.5). In-hospital mortality occurred in 13 of 133 patients (10%) (4/30 [13%] neonates). Postdischarge mortality occurred in 10 of 120 patients (8%) at a median of 298 days post-Ross. Post-Ross neoaortic dilatation occurred, peaking at 4 to 5 SDs above normal at 2 to 3 years before returning to near-baseline z-score at a median follow-up of 11.5 [6.4-17.4] years. Autograft/left ventricular outflow tract reintervention was required in 5 of 120 patients (4%) at a median of 10.3 [4.1-12.8] years. Freedom from moderate or greater neoaortic regurgitation was 86% at 15 years. ConclusionsNeonates and infants experience excellent postdischarge survival and long-term freedom from autograft reintervention and aortic regurgitation after the Ross. Neoaortic dilatation normalizes in this population in the long-term. Increased consideration should be given to Ross in neonates and infants with aortic valve disease.

Diagnosis, management and long term cardiovascular outcomes of phenotypic profiles in pulmonary hypertension associated with congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm resulting in herniation of viscera into the chest. This condition is characterized by pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. PH is a key component of the pathophysiology of CDH in neonates and contributes to morbidity and mortality. Traditionally, PH associated with CDH (CDH-PH) is thought to be secondary to increased pulmonary arterial resistance and vasoreactivity resulting from pulmonary hypoplasia. Additionally, there is increasing recognition of associated left ventricular hypoplasia, dysfunction and elevated end diastolic pressure resulting in pulmonary venous hypertension in infants with CDH. Thus, hemodynamic management of these infants is complex and cautious use of pulmonary vasodilators such as inhaled nitric oxide (iNO) is warranted. We aim to provide an overview of different phenotypic profiles of CDH associated PH and potential management options based on current evidence and pathophysiology.

Foramen ovale flap aneurysm in fetuses associated with and without heart defects: Prenatal diagnosis, imaging, in-utero hemodynamics, pregnancy, and postnatal outcomes.

Isolated redundant foramen ovale flap aneurysm (RFOA) in the absence of restrictive foramen ovale is believed to be a cause for pseudocoarctation of aorta since the impediment of blood flow to the left heart can be severe, resembling the picture of left ventricular hypoplasia with retrograde aortic flow. The primary objective of the study is to find whether RFOA is always a benign lesion. The main focus of the study is to share my experience in particular on fetuses having redundant foramen ovale flap aneurysm developing into coarctation of aorta and to study the associated factors. Retrospective study (January 2020 to June 2023). All fetuses with RFOA associated with and without congenital heart defects were included. Fetuses with restrictive foramen ovale and RFOA with single ventricle hearts were excluded. The imaging, in-utero hemodynamics, pregnancy, and postnatal outcomes with at least 3 months follow-up were presented. During the study period, a total of 1499 fetal echocardiography were performed. Twenty-two fetuses with RFOA were included. Fourteen fetuses had isolated RFOA and eight had associated abnormalities [extracardiac (n=5); intracardiac (n=2), Both (n=1)]. Genetic evaluation were performed only in fetuses with associated defects were normal. Postnatally all isolated RFOA fetuses had no aortic arch obstruction. Fetuses with associated aberrant right subclavian artery, isolated left superior vena cava, absent ductus venosus and ventricular septal defects developed aortic arch obstruction after birth. RFOA causes smallish left ventricle in fetuses with tetralogy of Fallot which recovered to normal size postnatally. Isolated RFOA can be benign, however, if it is associated with cardiac or extracardiac anomalies predominantly resulted in aortic arch obstruction. Though it is a cause for pseudocoarctation of aorta, through postnatal reassessment of aortic arch is mandatory. Careful search for intracardiac and systemic venous anomalies is recommended. It created confusion regarding adequacy of left ventricle when associated with congenital heart defects.

Kiosk 4R-TA-04 - Fetal CMR Reveals Left Ventricular Hypoplasia and Redistribution of the Circulation in a Fetal Sheep Model of Left Heart Hypoplasia

Kiosk 4R-TA-04 - Fetal CMR Reveals Left Ventricular Hypoplasia and Redistribution of the Circulation in a Fetal Sheep Model of Left Heart Hypoplasia

"NASCI case of the month: left ventricular apical hypoplasia".

Left ventricular apical hypoplasia is a rare congenital condition. It can cause nonspecific symptoms and can be accompanied by cardiac conduction system alterations such as bundle branch block, atrial flutter (AF) or atrial fibrillation. The diagnosis mostly is made by imaging.

Echocardiography: conotruncal anomaly: a case of common arterial trunk with intact ventricular septum and hypoplastic left heart complex with unbalanced pulmonary stenoses.

We describe the echocardiographic findings of a common arterial trunk with intact ventricular septum, mitral and left ventricular hypoplasia, atretic left ventricular outlet and bilateral, and unbalanced pulmonary artery stenoses.

Isolated left ventricular apical hypoplasia: Systematic review and analysis of the 37 cases reported so far.

Isolated left ventricular apical hypoplasia (ILVAH), also known as truncated left ventricle (LV), is a very unusual cardiomyopathy. It is characterised by a truncated, spherical, and non-apex forming LV. The true apex is occupied by the right ventricle. Due to the rarity of the disease, just a few case reports and limited case series have been published in the field. To analysing the so far 37 reported ILVAH cases worldwide. The electronic databases PubMed and Scopus were investigated from their establishment up to December 13, 2022. The majority of cases reported occurred in males (52.7%). Mean age at diagnosis was 26.1 ± 19.6 years. More than a third of the patients were asymptomatic (35.1%). The most usual clinical presentation was breathlessness (40.5%). The most commonly detected electrocardiogram changes were T wave abnormalities (29.7%) and right axis deviation with poor R wave progression (24.3%). Atrial fibrillation/flutter was detected in 24.3%. Echocardiography was performed in 97.3% of cases and cardiac MRI in 91.9% of cases. Ejection fraction was reduced in more than a half of patients (56.7%). An associated congenital heart disease was found in 16.2%. Heart failure therapy was administered in 35.1% of patients. The outcome was favorable in the vast majority of patients, with just one death. ILVAH is a multifaceted entity with a so far unpredictable course, ranging from benign until the elderly to sudden death during adolescence.

Biventricular isolated apical hypoplasia: First-time description of a new cardiac abnormality by multimodality imaging

Left ventricular isolated hypoplasia is a seldom-described cardiac abnormality. Right ventricular hypoplasia is usually associated with congenital anomalies of the pulmonary or the tricuspid valve, whereas biventricular isolated apical hypoplasia has never been described. We report the case of a 48-year-old man with no history of known cardiac disease who was found to have a complex cardiac abnormality characterized by: 1) Deficiency of the myocardium within the biventricular apex with adipose tissue infiltration; 2) Truncated right ventricle because of an absent trabecular portion of the inflow tract; 3) Truncated and spherical left ventricular apex; 4) Origin of the mitral papillary muscle in the flattened anterior left ventricular apex. Multimodality imaging was performed to delineate the morphological and functional characteristics of this cardiomyopathy fully. To the best of our knowledge, this is the first description of a new cardiac abnormality characterized by the hypoplasia of the apical region of both ventricles in the absence of valvular or coronary artery disease.

Aspects related to left ventricular hypoplasia, possibilities and outcomes of surgical treatments: an integrative literature review

Objective: To analyze the current scientific collection related to left ventricular hypoplasia, analyzing the possibilities and outcomes of surgical treatments. Methodology: Integrative literature review that searched for articles in the Virtual Health Library (VHL), using the descriptors: "left ventricular hypoplasia" AND "treatment" from 2020-2022. Results and discussion: The sample corpus totaled 23 articles, with 02 thematic axes: (I) Studies found and their surgical techniques and (II) Comparison of studies regarding patient survival. Final Considerations: There was a better understanding of the importance of surgical palliation for the viability of patient survival. More scientific evidence is suggested that raises greater knowledge about mortality between stages, as well as mortality from other procedures involved in palliative reconstruction, providing additional evidence for the long-term survival rate.

What Is the Hypoplastic Left Heart Syndrome?

As yet, there is no agreed definition for the so-called "hypoplastic left heart syndrome". Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a "syndrome" in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described "hypoplasia of the aortic outflow tract complex". In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.

Timing of umbilical cord clamping in infants with congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a severe birth anomaly where a defect in the diaphragm allows abdominal organs to herniate into the chest with compression of the intrathoracic structures, specifically the lungs and heart. Pulmonary and left ventricular hypoplasia result in respiratory insufficiency after birth with disordered transition and persistent pulmonary hypertension of the newborn (PPHN). As a result, infants need immediate intervention after birth to support the transition. Delayed cord clamping (DCC) is recommended for all healthy newborns and improves outcomes in infants born preterm and in infants with congenital heart disease; however, DCC may not be feasible in newborns needing immediate intervention after birth. Recent studies have explored resuscitation with intact umbilical cords, to determine feasibility, safety, and efficacy in infants with CDH, with promising results. In this report we discuss the physiologic basis for intact cord resuscitation in infants with CDH and review the above reports with a view to determining optimal timing of umbilical cord clamping in infants with CDH.

Appendiceal perforation: A rare case of massive neonatal pneumoperitoneum

A pre-term newborn male was admitted to the pediatric cardiac intensive care unit with severe left ventricular and aortic hypoplasia. On day-of-life-13, massive pneumoperitoneum was incidentally identified on a chest radiograph after central line placement. Emergent exploration with thorough examination revealed only a focal perforation at the appendiceal tip. Standard appendectomy was performed; pathology showed appendiceal perforation with focal mucosal ulceration without inflammation . Literature review demonstrated neonates with appendiceal perforation have nonspecific symptoms at presentation (emesis, poor feeding, abdominal erythema) which may be difficult to distinguish from other etiologies of perforation. Infants with concomitant cardiac pathology are at increased risk for spontaneous intestinal perforation due to malperfusion. However, appendiceal perforation does not lead the differential for neonatal massive pneumoperitoneum; rather, large volume pneumoperitoneum typically results from gastric perforation. The true incidence is unknown with fewer than 10 reported cases; importantly, the literature often lacks distinction between spontaneous-perforation versus appendicitis-with-perforation. Given its rarity, discrete examination of the appendix and appendiceal pathology may be overlooked when evaluating neonates with pneumoperitoneum during a standard of care operative exploration. Particularly in patients with high-risk comorbidities where gastric or ileal pathology is not appreciated after thorough exploration, appendiceal perforation should be considered.

Impaired Human Cardiac Cell Development due to NOTCH1 Deficiency.

NOTCH1 pathogenic variants are implicated in multiple types of congenital heart defects including hypoplastic left heart syndrome, where the left ventricle is underdeveloped. It is unknown how NOTCH1 regulates human cardiac cell lineage determination and cardiomyocyte proliferation. In addition, mechanisms by which NOTCH1 pathogenic variants lead to ventricular hypoplasia in hypoplastic left heart syndrome remain elusive. CRISPR (Clustered Regularly Interspaced Short Palindromic Repeats)/Cas9 genome editing was utilized to delete NOTCH1 in human induced pluripotent stem cells. Cardiac differentiation was carried out by sequential modulation of WNT signaling, and NOTCH1 knockout and wild-type differentiating cells were collected at day 0, 2, 5, 10, 14, and 30 for single-cell RNA-seq. Human NOTCH1 knockout induced pluripotent stem cells are able to generate functional cardiomyocytes and endothelial cells, suggesting that NOTCH1 is not required for mesoderm differentiation and cardiovascular development in vitro. However, disruption of NOTCH1 blocks human ventricular-like cardiomyocyte differentiation but promotes atrial-like cardiomyocyte generation through shortening the action potential duration. NOTCH1 deficiency leads to defective proliferation of early human cardiomyocytes, and transcriptomic analysis indicates that pathways involved in cell cycle progression and mitosis are downregulated in NOTCH1 knockout cardiomyocytes. Single-cell transcriptomic analysis reveals abnormal cell lineage determination of cardiac mesoderm, which is manifested by the biased differentiation toward epicardial and second heart field progenitors at the expense of first heart field progenitors in NOTCH1 knockout cell populations. NOTCH1 is essential for human ventricular-like cardiomyocyte differentiation and proliferation through balancing cell fate determination of cardiac mesoderm and modulating cell cycle progression. Because first heart field progenitors primarily contribute to the left ventricle, we speculate that pathogenic NOTCH1 variants lead to biased differentiation of first heart field progenitors, blocked ventricular-like cardiomyocyte differentiation, and defective cardiomyocyte proliferation, which collaboratively contribute to left ventricular hypoplasia in hypoplastic left heart syndrome.

Isolated Left Ventricular Apical Hypoplasia: A Very Rare Congenital Anomaly Characterized by Multimodality Imaging and Invasive Testing.

HomeCirculation: Cardiovascular ImagingAhead of PrintIsolated Left Ventricular Apical Hypoplasia: A Very Rare Congenital Anomaly Characterized by Multimodality Imaging and Invasive Testing No AccessResearch ArticleRequest AccessAboutView PDFSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsNo AccessResearch ArticleRequest AccessIsolated Left Ventricular Apical Hypoplasia: A Very Rare Congenital Anomaly Characterized by Multimodality Imaging and Invasive Testing Samuel D. Maidman, MD, William D. Salerno, MD, Dan G. Halpern, MD, Robert Donnino, MD and Muhamed Saric, MD, PhD Samuel D. MaidmanSamuel D. Maidman https://orcid.org/0000-0002-1376-5526 Leon H. Charney Division of Cardiology, NYU Grossman School of Medicine, NYU Langone Health. (S.D.M., D.G.H., R.D., M.S.) Search for more papers by this author , William D. SalernoWilliam D. Salerno Division of Cardiology, Hackensack University Medical Center, Hackensack Meridian School of Medicine, NJ (W.D.S.). Search for more papers by this author , Dan G. HalpernDan G. Halpern https://orcid.org/0000-0002-1656-798X Leon H. Charney Division of Cardiology, NYU Grossman School of Medicine, NYU Langone Health. (S.D.M., D.G.H., R.D., M.S.) Search for more papers by this author , Robert DonninoRobert Donnino Leon H. Charney Division of Cardiology, NYU Grossman School of Medicine, NYU Langone Health. (S.D.M., D.G.H., R.D., M.S.) Department of Radiology, NYU Grossman School of Medicine, NYU Langone Health. (R.D.) Division of Cardiology, Veteran Affairs NY Harbor Medical Center, Manhattan Campus (R.D.). Search for more papers by this author and Muhamed SaricMuhamed Saric Correspondence to: Muhamed Saric, MD, PhD, Adult Echocardiography Laboratory, 550 First Ave, New York, NY 10016. Email E-mail Address: [email protected] https://orcid.org/0000-0001-7682-3749 Leon H. Charney Division of Cardiology, NYU Grossman School of Medicine, NYU Langone Health. (S.D.M., D.G.H., R.D., M.S.) Search for more papers by this author Originally published30 Nov 2022https://doi.org/10.1161/CIRCIMAGING.122.014789Circulation: Cardiovascular Imaging. 2022;0:e014789FootnotesSupplemental Material is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.122.014789.For Sources of Funding and Disclosures, see page XXX.Correspondence to: Muhamed Saric, MD, PhD, Adult Echocardiography Laboratory, 550 First Ave, New York, NY 10016. Email muhamed.[email protected]org Previous Back to top Next FiguresReferencesRelatedDetails Advertisement Article InformationMetrics © 2022 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.122.014789PMID: 36448445 Originally publishedNovember 30, 2022 Keywordscongenitalisolated left ventricular apical hypoplasiamultimodality imagingPDF download Advertisement SubjectsCongenital Heart DiseaseImaging

Early Experience With Reverse Double Switch Operation for the Borderline Left Heart

This study reviews our early experience with the "reverse" double switch operation (R-DSO) for borderline left hearts. A retrospective review of children with borderline left hearts who underwent R-DSO between 2017 and 2021 was conducted. Patient characteristics and early hemodynamic and clinical outcomes were collected. R-DSO was performed in 8 patients with no operative or postoperative deaths; 5 underwent decompressing bidirectional Glenn. Left ventricular (LV) poor-compliance was the dominant pathophysiology. Four patients had undergone staged LV recruitment but were not candidates for anatomical biventricular circulation due to LV hypoplasia and/or diastolic dysfunction. 7/8 patients had risk factors for Fontan circulation including pulmonary vein stenosis, pulmonary hypertension, and pulmonary artery stenosis. Median age at R-DSO was 3.7 years (19 months-12 years). All patients were in sinus rhythm at discharge. At median follow-up of 15 months (57 days-4.1 years) no mortalities, reoperations or heart transplants had occurred. All patients had normal morphologic LV systolic function. In one patient, pre-existing pulmonary hypertension (HTN) resolved after R-DSO. Reinterventions included transcatheter mitral valve replacement for residual mitral stenosis and neo-pulmonary balloon valvuloplasty. In 4 patients follow-up catheterization done at a median of 519 days (320 days-4 years) demonstrated median cardiac index of 3.2 L/min/m2 (2.2-4); median sub-pulmonary left ventricular end diastolic pressure was 9 mm Hg (7-15); median inferior vena cava/baffle pressure was 8 mm Hg (7-13). R-DSO is an alternative to anatomical biventricular repair or single ventricle palliation in patients with borderline left hearts and can result in low inferior vena cava pressures and favorable early results. This approach can also relieve pulmonary HTN and allow future transplant candidacy.

Imaging of Left Ventricular Hypoplasia.

Left ventricular hypoplasia is a common finding in various forms of congenital heart disease. Echocardiography in the setting of left ventricular hypoplasia must comprehensively assess the size and function of all left-sided structures including the mitral valve, left ventricular outflow tract, aortic valve and aortic arch. Of most importance in any variation of left ventricular hypoplasia is the left ventricular inlet. In neonates, the left ventricular inlet often determines the adequacy of the left ventricle and is the most difficult component to treat surgically.