Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS)
Background: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare condition, encompassing approximately 1%–3% of congenital heart diseases. Patients with PA-IVS have functional single-ventricle physiology. The ultimate possible outcomes for patients with PA-IVS are biventricular circul...
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Fakultas Kedokteran, Universitas Diponegoro
2024-03-01
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| Series: | JAI (Jurnal Anestesiologi Indonesia) |
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| Online Access: | https://ejournal.undip.ac.id/index.php/janesti/article/view/53970 |
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| author | Ronald Jan Palenteng Riza Cintyandy |
| author_facet | Ronald Jan Palenteng Riza Cintyandy |
| author_sort | Ronald Jan Palenteng |
| collection | DOAJ |
| description | Background: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare condition, encompassing approximately 1%–3% of congenital heart diseases. Patients with PA-IVS have functional single-ventricle physiology. The ultimate possible outcomes for patients with PA-IVS are biventricular circulation, 1.5-ventricle or single-ventricle palliation, or cardiac transplantation. The bidirectional cavopulmonary shunt (BCPS) procedure directs flow from the superior vena cava into both the right and left pulmonary arteries, permitting flow to both lungs. The shunt is considered to be the second stage of palliation and is generally preparative for the third-stage Fontan procedure. The BCPS improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance.
Case: A 10-month-old, 25-day-old boy, weight 9.93 kg, body length 72 cm, diagnosed with PA-IVS, restricted persistent foramen ovale (PFO), right ventricle hypoplastic, and turtuous patent ductus arteriosus (PDA), underwent BCPS, atrial septectomy, and PDA stent evacuation surgery. The patient underwent a PDA stenting and ballooning atrial septectomy (BAS) at 17 days of age at the cathlab. The patient was cyanotic with stable hemodynamics and a saturation of 72% preoperatively.
Discussion: Preoperative fasting must be observed to maintain the patient's hydration state. Pulmonary blood flow and systemic blood flow must be balanced. An adequate analgetic can prevent pain stimuli that increase pulmonary vascular resistance. Drugs to reduce the afterload, such as milrinone, are needed. Mechanical ventilation was set to get PaCO2 between 40 and 45 mmHg. Maintain the normal heart beat, preload, and contractility to maintain cardiac output (CO) with saturation 80–85%. Wean from mechanical ventilation as soon as possible.
Conclusion: Anesthetic management for BCPS in patients with single ventricles from the preoperative period, intraoperative period, and postoperative period. Understanding single ventricle phsiology is important in order to treat the patient. |
| format | Article |
| id | doaj-art-d14c861a563c48709bce21d0df65b60a |
| institution | OA Journals |
| issn | 2337-5124 2089-970X |
| language | English |
| publishDate | 2024-03-01 |
| publisher | Fakultas Kedokteran, Universitas Diponegoro |
| record_format | Article |
| series | JAI (Jurnal Anestesiologi Indonesia) |
| spelling | doaj-art-d14c861a563c48709bce21d0df65b60a2025-08-20T02:35:54ZengFakultas Kedokteran, Universitas DiponegoroJAI (Jurnal Anestesiologi Indonesia)2337-51242089-970X2024-03-01161476410.14710/jai.v0i0.5397023375Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS)Ronald Jan Palenteng0Riza Cintyandy1Department of Anesthesiology and Intensive Care Unit, dr. H. Jusuf SK Hospital, Tarakan, IndonesiaDepartment of Anesthesiology and Post Operative Intensive Care Unit, Heart and Vascular Harapan Kita Hospital, Jakarta, IndonesiaBackground: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare condition, encompassing approximately 1%–3% of congenital heart diseases. Patients with PA-IVS have functional single-ventricle physiology. The ultimate possible outcomes for patients with PA-IVS are biventricular circulation, 1.5-ventricle or single-ventricle palliation, or cardiac transplantation. The bidirectional cavopulmonary shunt (BCPS) procedure directs flow from the superior vena cava into both the right and left pulmonary arteries, permitting flow to both lungs. The shunt is considered to be the second stage of palliation and is generally preparative for the third-stage Fontan procedure. The BCPS improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance. Case: A 10-month-old, 25-day-old boy, weight 9.93 kg, body length 72 cm, diagnosed with PA-IVS, restricted persistent foramen ovale (PFO), right ventricle hypoplastic, and turtuous patent ductus arteriosus (PDA), underwent BCPS, atrial septectomy, and PDA stent evacuation surgery. The patient underwent a PDA stenting and ballooning atrial septectomy (BAS) at 17 days of age at the cathlab. The patient was cyanotic with stable hemodynamics and a saturation of 72% preoperatively. Discussion: Preoperative fasting must be observed to maintain the patient's hydration state. Pulmonary blood flow and systemic blood flow must be balanced. An adequate analgetic can prevent pain stimuli that increase pulmonary vascular resistance. Drugs to reduce the afterload, such as milrinone, are needed. Mechanical ventilation was set to get PaCO2 between 40 and 45 mmHg. Maintain the normal heart beat, preload, and contractility to maintain cardiac output (CO) with saturation 80–85%. Wean from mechanical ventilation as soon as possible. Conclusion: Anesthetic management for BCPS in patients with single ventricles from the preoperative period, intraoperative period, and postoperative period. Understanding single ventricle phsiology is important in order to treat the patient.https://ejournal.undip.ac.id/index.php/janesti/article/view/53970anesthetic managementbidirectional cavopulmonary shunt (bcps)intact ventricular septumpulmonary atresiasingle ventricle |
| spellingShingle | Ronald Jan Palenteng Riza Cintyandy Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS) JAI (Jurnal Anestesiologi Indonesia) anesthetic management bidirectional cavopulmonary shunt (bcps) intact ventricular septum pulmonary atresia single ventricle |
| title | Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS) |
| title_full | Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS) |
| title_fullStr | Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS) |
| title_full_unstemmed | Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS) |
| title_short | Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS) |
| title_sort | anesthesia in a patient with pulmonary atresia with intact ventricular septum pa ivs underwent bidirectional cavo pulmonary shunt bcps |
| topic | anesthetic management bidirectional cavopulmonary shunt (bcps) intact ventricular septum pulmonary atresia single ventricle |
| url | https://ejournal.undip.ac.id/index.php/janesti/article/view/53970 |
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