Humans ; Hypoplastic Left Heart Syndrome

No abstract available.
Humans ; Hypoplastic Left Heart Syndrome* ; Survivors*

A patch design using bovine pericardial patch for aortic enlargement in the Norwood procedure has been introduced to avoid problems related to homograft availability. We report 2 successful cases of Norwood procedure with home-made bovine pericardial patch. The first case was a 23-day-old (2.2 kg) patient with multi-level left ventricular outflow tract obstruction with ductal-dependent systemic circulation. The other case was a 9-day-old (3 kg) patient with hypoplastic left heart syndrome. This technique was relatively easy to perform, reproducible and quite effective like homograft patch.
Allografts ; Humans ; Hypoplastic Left Heart Syndrome ; Norwood Procedures*

Hypoplastic left heart syndrome is a rare congenital heart disease. This is a universally fatal leison, and approximately 80% of patients die within the first week of life. The diagnosis should be considered in infants, with the sudden onset of heart failure, systemic hypoperfusion and cyanosis. 2 cases of hypoplastic left syndrome are presented with review of related literatures.
Cyanosis ; Diagnosis ; Heart Defects, Congenital ; Heart Failure ; Humans ; Hypoplastic Left Heart Syndrome* ; Infant

In the last decade the hybrid procedure has emerged as an alternative stage I palliation in neonates with hypoplastic left heart syndrome (HLHS). This review discusses the historical aspect, surgical and interventional techniques, current outcomes and future direction of this procedure. Hybrid palliation yields equivalent but not superior stage I palliation survival and comparable 1-year survival to conventional Norwood palliation, comparable prestage II hemodynamics and pulmonary artery growth, and preserved ventricular function in stage II palliation. Hybrid palliation utilizes significantly less resource and shortens postoperative recovery. In comprehensive stage II palliation the impact of pulmonary artery reconstruction on subsequent pulmonary artery growth has not been determined and should be further investigated. A prospective, randomized trial is warranted to compare these two surgical strategies for neonates with hypoplastic left heart syndrome.
Chimera ; Heart ; Hemodynamics ; Humans ; Hypoplastic Left Heart Syndrome ; Infant, Newborn ; Pulmonary Artery ; Stents ; Ventricular Function

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.
Aorta ; Aorta, Thoracic* ; Esophagus ; Heart Defects, Congenital ; Humans ; Hypoplastic Left Heart Syndrome* ; Norwood Procedures* ; Pulmonary Artery ; Subclavian Artery ; Trachea

A 12-day-old female baby underwent a Norwood procedure for hypoplastic left heart syndrome. The left superior vena cava (LSVC), which was found incidentally during the operation, was divided to facilitate surgical exposure. After the operation, she developed signs of low cardiac output and died 7 hours afterward. Autopsy findings showed that the coronary sinus was atretic at the orifice without unroofing into both atria, rendering the LSVC the sole route of coronary sinus drainage. In patients with incidentally-found LSVC during surgery, special care should be taken to leave the LSVC intact because the LSVC may be the exclusive drainage vein of the coronary venous system.
Autopsy ; Cardiac Output, Low ; Coronary Sinus ; Drainage ; Female ; Humans ; Hypoplastic Left Heart Syndrome ; Norwood Procedures ; Veins ; Vena Cava, Superior

OBJECTIVETo analyze the clinical and cardiac imaging characteristics of patients with left ventricular apical hypoplasia (LVAH).

METHODSFrom January 2008 to January 2012, seven patients [3 male/4 female, age: 6 - 44 (19.9 ± 14.2) years] with LVAH were included in this cohort. Transthoracic echocardiogram was performed in all patients, cardiovascular MRI was performed in 3 patients and cardiovascular CT in another 2 patients. In addition, one LVAH patient underwent cardiac catheterization and angiography examination.

RESULTSFour out of 7 patients complained chest discomfort. Precordial murmur was heard in 3 patients. Atrial fibrillation was evidenced by electrocardiogram in 3 patients. Left ventricular end-diastolic diameter [(57.9 ± 11.6) mm] increased while left ventricule (LV) longitudinal diameter reduced in all patients. Left ventricular systolic function was reduced in 2 patients and mean LVEF was (47.6 ± 17.2)%. The interventricular septum bulged towards the right, and the ventricular septum thickness was (7.3 ± 1.2) mm. The papillary muscles were dominant on the flattened LV anteroapical region. The right ventricle elongated and wrapped around the hypoplastic left ventricular apex, and the dimension of right ventricle was (19.7 ± 7.6) mm. Focal fat replacement of the left ventricular apical wall was evidenced in 5 patients underwent cardiovascular MRI or CT examinations.

CONCLUSIONSClinical symptoms are non-specific in LVAH patients. Truncated and spherical LV, abnormal origin of papillary muscles in the flattened LV anterior apex and an elongated right ventricle wrapping around the LV apex as well as focal fat replacement of the left ventricular apical wall are typical imaging characteristics of LVAH.

Adolescent ; Adult ; Child ; Diagnostic Imaging ; Female ; Humans ; Hypoplastic Left Heart Syndrome ; diagnosis ; diagnostic imaging ; Male ; Ultrasonography ; Young Adult

Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe human fetal heart in-utero and to diagnose congenital heart disease prenatally over the last 20 years. The diagnosis of HLHS in fetal echocardiography is based on 2-dimensional echocardio -graphic evidence of a diminutive ascending aorta, aortic atresia, mitral atresia or severe stenosis and a hypoplastic left ventricle. Abnormal flow direction through atrial septum or through isthmus greatly aids the diagnosis. This report shows a fetal case who showed hypoplastic left side chambers and retrograde isthmic flow and was diagnosed with hypoplastic left heart syndrome. After birth, although the baby had tachy-dyspnea for the first 3 weeks, she finally recovered without any intervention and showed catch up growth of left side chambers. This case illustrates the extreme difficulty of assessing left ventricle in a fetus.
Aorta ; Aortic Valve Stenosis ; Atrial Septum ; Constriction, Pathologic ; Diagnosis* ; Echocardiography ; Fetal Heart ; Fetus* ; Heart ; Heart Defects, Congenital ; Heart Ventricles ; Humans ; Hypoplastic Left Heart Syndrome* ; Parturition

Over the last several decades there has been a remarkable change in the therapeutic strategy of congenital heart disease. Development of new tools and devices, accumulations of experience, technical refinement have positively affected the outcome of interventional treatment. Many procedures including atrial septostomy, balloon valvuloplasty, balloon dilation of stenotic vessel with or without stent implantation, transcatheter occlusion of abnormal vascular structure, transcatheter closure of patent arterial duct and atrial septal defect, are now performed as routine interventional procedures in many institutes. In diverse conditions, transcatheter techniques also provide complementary and additive role in combination with surgery. Intraoperative stent implantation on stenotic vessels, perventricular device insertion, and hybrid stage 1 palliative procedure for hypoplastic left heart syndrome have been employed in high risk patients for cardiac surgery with encouraging results. Transcatheter closure of ventricular septal defect has been performed safely showing comparable result with surgery. Investigational procedures such as percutaneous valve insertion and valve repair are expected to replace the role of surgery in certain group of patients in the near future. Continuous evolvement in this field will contribute to reduce the risk and suffering from congenital heart disease, while surgery will be still remained as a gold standard for significant portion of congenital heart disease.
Academies and Institutes ; Balloon Valvuloplasty ; Catheterization ; Embolization, Therapeutic ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Hypoplastic Left Heart Syndrome ; Stents ; Thoracic Surgery