Advances in diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease

Suesaowalak, Monnipa; Cleary, John P.; Chang, Anthony

doi:10.1007/s12519-010-0002-9

Cited by 25 publications

(28 citation statements)

References 135 publications

(167 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…PH, artmış pulmoner kan akımının olduğu konjenital kalp anomalilerinde sıklıkla görülür ve tam düzeltme ameliyatı sonrası bazı hastalarda pulmoner hipertansif kriz, sağ kalp yetersizliği, arteriyal desatürasyon, ani kardiyak arrrest gibi yüksek mortalite ile seyreden sorunlara yol açabilir. Pulmoner hipertansif atak geliştiğinde, pulmoner vazokonstriksiyonla birlikte sağ ventrikül basıncında ani bir yükselme olmakta, hastada ani olarak kardiyopulmoner arrest gelişmektedir (3)(4)(5)(6) . Hastamızda olduğu gibi, sedasyonun kesilmesi sonrasında weaning periyodunda ani kardiyopulmoner arrest gelişmiş ve sonrasında hastaya başarılı bir resüsitasyon yapılabilmiştir.…”

Section: Discussionunclassified

“…KKH'de, pulmoner kan akımının artması sonucunda endotel hasarının oluşması PH gelişiminde önemli rol oynar (3,4) . Endotel hasarı sonucunda pulmoner vasküler reaktivite ve pulmoner vasküler rezistans (PVR) artar; bu da, akut sağ kalp yetmezliği, dolaşım kollapsı ve ölümle sonuçlanabilir (5) .…”

Section: Introductionunclassified

“…Tedavide sedasyon, yüksek fraksiyone oksijen ile ventilasyon, pozitif inotropik destek, asidozdan kaçınma gibi yöntemlerin yanında birçok medikal tedavi geliştirilmiştir (3,4) .…”

Section: Introductionunclassified

“…Bir prostasiklin (Prostoglandin I2) analoğu olan iloprost, pulmoner yatakta daha spesifik ve anlamlı vazodilatasyon yapar, sistemik vasküler rezistansı (SVR) belirgin bir şekilde düşürmeden PVR'yi azaltarak PH tedavisinde kullanılır (4) .…”

Section: Introductionunclassified

See 3 more Smart Citations

Pulmonary Hypertension Crisis in Pediatric Patient Undergoing Congenital Heart Surgery: Case Report

Altun¹,

Balci²,

Özker³

et al. 2014

GKDA Derg

View full text Add to dashboard Cite

Section: Discussionunclassified

Section: Introductionunclassified

“…Tedavide sedasyon, yüksek fraksiyone oksijen ile ventilasyon, pozitif inotropik destek, asidozdan kaçınma gibi yöntemlerin yanında birçok medikal tedavi geliştirilmiştir (3,4) .…”

Section: Introductionunclassified

See 2 more Smart Citations

Pulmonary Hypertension Crisis in Pediatric Patient Undergoing Congenital Heart Surgery: Case Report

Altun¹,

Balci²,

Özker³

et al. 2014

GKDA Derg

View full text Add to dashboard Cite

“…PAH may develop in congenital heart defects with increased pulmonary flow (especially VSD, complete atrioventricular septal defect, aortopulmonary window) when high systemic pressure is transmitted to the pulmonary circulation [3]. D-TGA with VSD is a special condition predisposing to obstructive pulmonary vascular disease.…”

Section: Review Of Literaturementioning

confidence: 99%

Senning operation as a palliative therapy for a girl with complex heart defect and Eisenmenger syndrome (RCD code: II-1A.4d)

Kuźma

Rudziński

Kopeć

et al. 2016

Journal of Rare Cardiovascular Diseases

View full text Add to dashboard Cite

Development of irreversible pulmonary hypertension in D-transposition of the great arteries (D-TGA) with ventricular septal defect (VSD) is a well-known phenomenon. Coexistence of left ventricular outflow tract obstruction (LVOTO) should theoretically protect the patient against pulmonary arterial hypertension (PAH). Application of the Blalock-Taussig shunt (B-TS) temporarily improves condition of the patient, but may contribute to irreversible PAH requiring palliative complex therapy. We present a case of a 18 year-old female with congenital complex heart defect: D-TGA with inflow VSD and subpulmonary stenosis, Eisenmenger syndrome, who was treated with several subsequent palliative cardiosurgical operations, including B-TS and Senning procedure and eventually, combined PAH- CASE REPORTRare congenital cardiovascular diseases Journal of Rare Cardiovascular Diseases 2016; 3 (1): 14-16 www.jrcd.eu Case presentationWe present a case of an 18 year-old female with a complex congenital heart defect and Eisenmenger syndrome treated with palliative cardiosurgical operations and pharmacotherapy for pulmonary arterial hypertension (PAH).The patient was delivered at term with body weight of 3700 g. Central cyanosis and soft systolic murmur with loudness of 2/6 in Levine scale were present at birth. Peripheral oxygen saturation (SpO 2 ) was very low (60-70%) with capillary blood partial oxygen pressure (pO 2 ) of 27 mm Hg. Transthoracic echocardiography (TTE) showed complex congenital heart defect: D-transposition of the great arteries (D-TGA), large inlet ventricular septal defect (VSD) with tricuspid valve (TV) straddling, patent foramen ovale (PFO) and sub-pulmonary stenosis (PS) due to the accessory connective tissue strands. Prostaglandin E1 (PGE1) infusion was started. Subsequent TTE study revealed moderate left ventricular outflow tract obstruction (LVOTO) with pressure gradient of 50 mmHg and no left-to-right shunt through the PFO.The child was qualified for Blalock-Taussig shunt (B-TS) at the age of 4 weeks. After the operation SpO 2 increased to 75-80% with pO 2 of 36 mm Hg. The patient was discharged home in a satisfactory clinical condition.At the age of 15 months cyanosis became intense with a drop of saturation to 60-70%. TTE showed D-TGA, large inlet VSD, mild LVOTO (pressure gradient of 17 mm Hg), extremely dilated pulmonary trunk and occluded B-TS. Cardiac catheterization revealed severe pulmonary hypertension (PH) with high pulmonary vascular resistance (PVR) of 10 Wood units (WU) and abnormal pulmonary-to-systemic resistance ratio (Rp/Rs) of 0.6 (Table 1, 2) unresponsive to 100% oxygen inhalation test. The patient was qualified for atrial septectomy to facilitate mixing of the blood at the atrial level. However, no improvement was observed after the operation. Therefore, the patient was qualified for palliative Senning procedure which was performed at the age of 29 months. During the operation a baffle within the atria was made in order to reroute

show abstract

Two Cases of Pulmonary Hypertension Associated with Type III Glycogen Storage Disease

et al. 2011

View full text Add to dashboard Cite

Glycogen storage diseases (GSDs) comprise a large, heterogeneous group of disorders characterized by abnormal glycogen deposition. Multiple cases in the literature have demonstrated an association between GSD type I and pulmonary arterial hypertension (PAH). We now also report on two patients with GSD type III and PAH, a novel association. The first patient was a 16-year-old girl of Nicaraguan descent with a history of hepatomegaly and growth retardation. Molecular testing identified a homozygous 17delAG mutation in AGL consistent with GSD type IIIb. At the age of 16, she was found to have PAH and was started on medical therapy. Two years later, she developed acute chest pain and died shortly thereafter. The second patient is a 13-year-old girl of Colombian descent homozygous for the c.3911dupA mutation consistent with GSD IIIa. An echocardiogram at age 2 showed left ventricular hypertrophy, which resolved following the institution of a high protein, moderate carbohydrate diet during the day and continuous gastric-tube feeding overnight. At the age of 12, she was found to have pulmonary hypertension. She was started on sildenafil, and her clinical status has shown marked improvement including normalization of her elevated transaminases. PAH may be a rare association in patients with GSD IIIa and IIIb and should be evaluated with screening echocardiograms for cardiac hypertrophy or if they present with symptoms of right-sided heart failure such as shortness of breath, chest pain, cyanosis, fatigue, dizziness, syncope, or edema. Early diagnosis of PAH is important as increasingly effective treatments are now available.

show abstract

Advances in diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease

Cited by 25 publications

References 135 publications

Pulmonary Hypertension Crisis in Pediatric Patient Undergoing Congenital Heart Surgery: Case Report

Pulmonary Hypertension Crisis in Pediatric Patient Undergoing Congenital Heart Surgery: Case Report

Senning operation as a palliative therapy for a girl with complex heart defect and Eisenmenger syndrome (RCD code: II-1A.4d)

Two Cases of Pulmonary Hypertension Associated with Type III Glycogen Storage Disease

Contact Info

Product

Resources

About