Hypoplastic left heart syndrome: multiprofessional follow-up in the mid-term following palliative procedures

1997 ◽  
Vol 7 (3) ◽  
pp. 248-253 ◽  
Author(s):  
Petter S. Hagemo ◽  
Magnhild Rasmussen ◽  
Grete Bryhn ◽  
Inger Helena Vandvik
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Motor Development ◽  
Autistic Traits ◽  
Attention Problems ◽  
Child Psychiatrist ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Mild Degree ◽  
The Right ◽  
Left Heart Syndrome

AbstractOf the 12 children who represent the complete cohort of Norwegian patients surviving the complete palliative operative programme for hypoplastic left heart syndrome, 10 were studied by a pediatric cardiologist, a pediatric neurologist, a neuropsychologist and a child psychiatrist. We found significant morbidity. Hemodynamic function was relatively uncompromised in eight of the patients, but one had significant cyanosis due to use of an operative technique subsequently abandoned and one had obstruction of the flow of blood from the left to the right atrium. Five children had disturbances of rhythm, with one requiring insertion of a pacemaker. One child had epilepsy. Delayed motor development, most often to mild degree, was found in seven children, while six had attention problems. Mental retardation was found in two children and autistic traits in one. Despite these various problems, the parents were seemingly satisfied with their own lives, and the quality of life of their children.

Pathological changes and myocardial remodelling related to the mode of shunting following surgical palliation for hypoplastic left heart syndrome

2008 ◽  
Vol 18 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Massimo A. Padalino ◽  
Chiara Castellani ◽  
Silvia Toffoli ◽  
Mila Della Barbera ◽  
Ornella Milanesi ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Pulmonary Shunt ◽  
Hypoplastic Left Heart ◽  
Surgical Palliation ◽  
The Right ◽  
Left Heart Syndrome

AbstractBackgroundThe modification of placing the shunt from the right ventricle to the pulmonary arteries, also known as Sano procedure, has allegedly improved results over the short term in surgical palliation of hypoplastic left heart syndrome with the Norwood procedure. With this in mind, we reviewed autopsied specimens from neonates and children who did not survive after either a classic arterio-pulmonary shunt, or the modified procedure with the shunt placed from the right ventricle to the pulmonary arteries, so as to evaluate the pathological substrates of the remodelling of the systemic right ventricle, assessing any differences induced by the 2 techniques.MethodsWe obtained the hearts from 11 patients with neonatal diagnosis of hypoplastic left heart syndrome who died after the first or second stages of the Norwood sequence of operations, comparing them with 6 normal hearts matched for age and weight. Macroscopic, microscopic and morphometric analysis were performed on each specimen, evaluating the diameter of the myocytes, extracellular matrix remodelling in terms of fibrosis and type of collagen, and vascularization in terms of capillary density.ResultsHypertrophy of the myocytes was significantly increased in the hearts from patients having either a classic arterio-pulmonary or the ventriculo-pulmonary modification of the shunt compared to controls (p < 0.05). Myocardial fibrosis was increased in those having a shunt placed from the right ventricle to the pulmonary arteries when compared to the other 2 groups. The ratio of collagen I to collagen III was similar in those undergoing a classic arterio-pulmonary shunt compared to controls (0.94), but was lower in those having a shunt placed from the right ventricle to the pulmonary arteries (0.61), with an increase in collagen type III. The density of capillaries was lower in those who had undergone a classic arterial shunt when compared to the others.ConclusionWe have shown greater remodelling of the ventricular myocardial extracellular matrix in patients having a shunt from the right ventricle to the pulmonary arteries when compared to those having a classic arterio-pulmonary shunt, with this remodelling progressing even after the neonatal period. This may influence a later suboptimal ventricular performance.

Hypoplastic left heart syndrome with anomalous origin of right pulmonary artery

2020 ◽  
pp. 021849232095506
Author(s):  
Ashish Mishra ◽  
Bhavik Champaneri ◽  
Yashpal Rana ◽  
Gaurav Singh ◽  
Senthilraj Thangasami ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Venous Drainage ◽  
Anomalous Origin ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Right Pulmonary Artery ◽  
Complex Forms ◽  
The Right ◽  
Left Heart Syndrome

Classic hypoplastic left heart syndrome is a rare but fatal congenital heart disease associated with variable underdevelopment of the left side of the heart. Complex forms of hypoplastic left heart syndrome have been reported to coexist with anomalous pulmonary venous drainage, transposition of the great arteries, or pulmonary valve dysplasia. We report a case of hypoplastic left heart syndrome with anomalous origin of the right pulmonary artery from the ascending aorta a rare association not reported in the literature. Preoperative comprehensive echocardiography is essential for diagnosis and accurate recognition of such rare anatomic variations.

scholarly journals Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

2020 ◽  
Vol 57 (6) ◽  
pp. 1098-1104
Author(s):  
Katarzyna Januszewska ◽  
Pawel Nawrocki ◽  
Anja Lehner ◽  
Julia Stegger ◽  
Felix Kleinerueschkamp ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Fontan Operation ◽  
Pulmonary Arteries ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Second Stage ◽  
The Right ◽  
Left Heart Syndrome ◽  
The Impact

Abstract OBJECTIVES The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3–9.8) years and median weight 12.7 (range 7.6–26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.

scholarly journals New Aspects in the Diagnosis and Therapy of Fetal Hypoplastic Left Heart Syndrome

2019 ◽  
Vol 79 (08) ◽  
pp. 863-872 ◽  
Author(s):  
Oliver Graupner ◽  
Christian Enzensberger ◽  
Roland Axt-Fliedner
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Treatment Options ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Non Invasive ◽  
New Findings ◽  
Parental Counseling ◽  
The Right ◽  
Systemic Ventricle ◽  
Left Heart Syndrome

AbstractFetal hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease with a lethal prognosis without postnatal therapeutic intervention or surgery. The aim of this article is to give a brief overview of new findings in the field of prenatal diagnosis and the therapy of HLHS. As cardiac output in HLHS children depends on the right ventricle (RV), prenatal assessment of fetal RV function is of interest to predict poor functional RV status before the RV becomes the systemic ventricle. Prenatal cardiac interventions such as fetal aortic valvuloplasty and non-invasive procedures such as maternal hyperoxygenation seem to be promising treatment options but will need to be evaluated with regard to long-term outcomes. Novel approaches such as stem cell therapy or neuroprotection provide important clues about the complexity of the disease. New aspects in diagnostics and therapy of HLHS show the potential of a targeted prenatal treatment planning. This could be used to optimize parental counseling as well as pre- and postnatal management of affected children.

A Rare Case of Embolic ST-Elevation Myocardial Infarction in an Adult Patient With Repaired Hypoplastic Left Heart Syndrome

2016 ◽  
Vol 8 (4) ◽  
pp. 543-549 ◽  
Author(s):  
Razan Shamoon ◽  
Habib Habib ◽  
Upamanyu Rampal ◽  
Aiman Hamdan ◽  
Mahesh Bikkina ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
St Segment Elevation ◽  
Catheterization Laboratory ◽  
Cardiac Catheterization Laboratory ◽  
Timi Grade ◽  
St Segment ◽  
The Right ◽  
Left Heart Syndrome

A 24-year-old male with past medical history of hypoplastic left heart syndrome and staged reconstructive surgery in infancy culminating in the Fontan circulation presented to the hospital with a chief complaint of chest pain described as an “elephant sitting” on his chest. Initial 12-lead electrocardiogram revealed 2-mm ST segment elevation in inferior leads, 3-mm ST-segment elevation in anterolateral precordial leads V3 and V4, and 2-mm ST-segment elevation in V5 and V6, with right axis deviation. He was transported emergently to the cardiac catheterization laboratory where coronary angiography revealed complete occlusion of multiple anomalous branches of the right coronary system with hazy appearance suggesting the presence of thrombotic material. An aspiration catheter was used successfully to reestablish TIMI grade III flow. The patient was treated with aspirin, brilinta (ticagrelor), and anticoagulation with vitamin K antagonism to prevent recurrent thromboembolic complications.

scholarly journals Choice of shunt type for the Norwood I procedure: does it make a difference?

2019 ◽  
Vol 30 (4) ◽  
pp. 630-635
Author(s):  
Keti Vitanova ◽  
Stanimir Georgiev ◽  
Rüdiger Lange ◽  
Julie Cleuziou
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Shunt Failure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Cross Sectional ◽  
The Difference ◽  
The Right ◽  
Left Heart Syndrome ◽  
Related Mortality ◽  
Blalock Taussig Shunt

Abstract OBJECTIVES This study aimed to compare pulmonary artery (PA) growth between patients who received a right ventricle-to-PA (RV–PA) shunt and those who received a modified Blalock–Taussig shunt (mBTS). METHODS All consecutive patients with hypoplastic left heart syndrome who underwent the Norwood I procedure between 2001 and 2017 were included in the study. Pre-stage 2 angiograms were analysed to measure the size of the PA. The Nakata index was calculated to estimate PA growth. The ratio of the right PA to left PA cross-sectional area (RPA/LPA) was used to calculate the difference in growth between the 2 branches. Study end points were shunt failure, shunt-related mortality and growth of the PAs. RESULTS A total of 223 patients with hypoplastic left heart syndrome (RV–PA group = 137, mBTS group = 86) underwent the Norwood I procedure, and 186 patients (RV–PA n = 116, mBTS n = 70) achieved the stage 2 procedure. PA growth was better in patients with mBTS (Nakata index: RV–PA = 282, mBTS = 315 mm2/m2, P = 0.021). LPA growth was worse compared to RPA growth in both groups (RPA/LPA: RV–PA = 1.21, mBTS = 1.29, P = 1.0). Patients with RV–PA shunts experienced more frequent shunt stenosis compared to patients with mBTS (26 vs 2, P &lt; 0.010). Freedom from shunt failure was 83.3 ± 3.2% and 94 ± 2% at 6 months in the RV–PA and mBTS groups, respectively (P = 0.003). CONCLUSIONS PA growth is significantly better in patients who received an mBTS. Moreover, patients with an RV–PA shunt more frequently experienced shunt failure due to shunt stenosis. However, survival after the NW procedure is not shunt dependent and growth of the LPA is less pronounced than RPA, regardless of the shunt type.

Implantation of stents as an alternative to reoperation in neonates and infants with acute complications after surgical creation of a systemic-to-pulmonary arterial shunt

2008 ◽  
Vol 18 (2) ◽  
pp. 177-184 ◽  
Author(s):  
Michael Kaestner ◽  
Ronald P. Handke ◽  
Joachim Photiadis ◽  
Matthias Sigler ◽  
Martin B.E. Schneider
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Interventional Procedure ◽  
Pulmonary Arteries ◽  
Emergency Situation ◽  
Interventional Treatment ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Acute Complications ◽  
The Right ◽  
Left Heart Syndrome

AbstractCreation of a systemic-to-pulmonary shunt is still the firstline treatment in neonates with duct-dependent pulmonary circulation, or in patients with hypoplastic left heart syndrome as a part of the first stage of the Norwood sequence. Acute complications after such surgery, such as stenosis, thrombosis, or kinking, are potentially lifethreatening. These complications require immediate revision or exchange of the shunt. In this report, we discuss interventional treatment as an alternative to surgery in 5 patients with acute stenosis or complete occlusion of a shunt.The age of the patients ranged from 12 to 62 days, with a median of 30 days, and their weights ranged between 2.2 and 4.6 kilogrammes, with a median of 3.2 kilogrammes. In 3 patients, the shunts were central or of modified Blalock-Taussig type, while the 2 patients with hypoplastic left heart syndrome had shunts from the right ventricle to the pulmonary arteries. We implanted 6 coronary arterial and 2 peripheral stent systems. The diameter of the balloon used for implantation had a ratio to the shunt of 0.9. to 1.All shunts were successfully reopened by stenting. During follow-up, 3 patients underwent further procedures on an elective basis. We have one patient on the waiting list for further surgical intervention, but one patient died of septicaemia unrelated to the interventional procedure one month after implantation.In our limited experience, implantation of stents is an effective and long-lasting treatment for complications of shunts in an emergency situation.

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